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MKSAP > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Skin manifestations of SLE

A

Acute cutaneous lupus erythematosus: malar or butterfly rash; erythema of cheeks and bridge of nose, +/- forehead & chin; spares nasolabial folds

Subacute cutaneous lupus erythematosus: photosensitive rash that occurs over the arms, neck, and face; may leave the skin with post-inflammatory changes (hyper- or hypo-pigmentation)

Discoid lupus erythematosus: most common chronic skin manifestation, isolated development of DLE usually does not mean the patient will develop SLE, most commonly face, neck, scalp, can cause scarring, atrophy, and permanent alopecia (as opposed to the other two above)

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2
Q

MSK involvement of SLE

A

Joint involvement: very common, non-erosive, small & large joint, but can cause subluxation and deformities

Osteonecrosis of the hips: may be attributed to steroid use especially prednisone doses of >20/d, sometimes vasculitis

Myalgia: common, but myositis is fairly rare; SLE myositis resembles polymyositis clinically and histologically; antimalarials and steroids can also cause myopathies

Fibromyalgia: is fairly common, must be distinguished

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3
Q

Kidney involvement in SLE

A

Lupus nephritis: occurs in about 70%, ass’d with anti-DS-DNA

Renal artery or vein thrombosis: in patients with APS or nephrotic syndrome

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4
Q

Neuro involvement in SLE

A

Note: Almost any involvement can occur; estimated that 70% have a neuropsych condition

Most common: headache, cognitive dysfunction, mood disorder

Central involvement: aseptic meningitis, demyelination, chorea, myelopathy, HA, mood disorder

Peripheral involvement: guillan-barre, peripheral neuropathy, mononeuropathy, myasthenia, etc

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5
Q

Indication for kidney biopsy in SLE

A

Increasing serum creatinine without explanation

Proteinuria >1000 mg/24 h

Proteinuria >500 mg/24 h with hematuria

Proteinuria >500 mg/24 h with cellular casts

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6
Q

How often should you monitor kidney in SLE?

A

Minor abnormalities (protein < 500/24h, minimal blood, no casts): monitor q3mo’s

Casts or significant changes: “further evaluation” (ie biopsy?)

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7
Q

Antibodies associated with SLE neuro disease?

A

Antineuronal

anti-NMDA receptor

antiribosomal P

antiphospholipid antibodies/lupus anticoagulant (APLA/LAC)

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8
Q

Cardiovascular involvement in SLE

A

Pericarditis & myocarditis: can be dramatic with acute-onset heart failure, EF often depressed maybe to < 20%, a rapid response to steroids supports SLE as the cause

Leibman-Sacks (sterile endocarditis)

Ischemic heart disease: increased risk with severe disease or prednisone dose >20/d

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9
Q

Pulmonary involvement in SLE

A

Pleuritis

Pleural effusion

Interstitial lung disease (actually rare in SLE)

Acute lupus pneumonitis (high mortality, tx = aggressive immunosuppression)

Diffuse alveolar hemorrhage (high mortality, tx = aggressive immunosuppression)

Shrinking lung syndrome (progressive lung volume loss; cause unknown; tx = agg immunosuppression)

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10
Q

Heme involvement in SLE

A

Any cytopenia

Hemolytic anemia

Antiphospholipid antibodies/lupus anticoagulant

Evans syndrome: severe thrombocytopenia + hemolytic anemia

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11
Q

GI involvement in SLE

A

GERD

Esophageal dysmotility

Sterile peritonitis

Mesenteric vasculitis (often with cutaneous vasculitis)

Mesenteric thrombosis (with APS)

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12
Q

Relation of SLE to malignancy

A

Increased risk of NHL, esp DLBCL, and cervical cancer

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13
Q

Clinical manifestations of RA

A

Joints: Multiple small joints of hands and feet, morning stiffness lasting at least 1 hour, DIP involvement is RARE, protracted onset (should be at least 6 weeks) +/- constitutional symptoms

Skin: Rheumatoid nodules, pyoderma gangrenosum, vasculitis (livedo reticularis & digital infarcts)

Eyes: Keratoconjunctivitis sicca, episcleritis, and vision-threatening inflammation (scleritis, uveitis, ulcerative keratitis, corneal filamentary keratitis

Pulmonary: Pleuritis, ILD (esp in smokers)

Cardiac: Pericarditis, rarely can develop restrictive cardiomyopathy not responsive to steroids

Other: Felty syndrome, mesangioproliferative GN, amyloidosis, alantoaxial subluxation, peripheral neuropathy

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14
Q

Diagnosis of RA

A

Need >= 6 points needed for “definite RA”:

Joint involvement (1-5 points; more for small & many joints)

Serology (3 for high-positive, 1 for low)

Acute phase reactants (1 pt for either ESR or CRP)

Duration of joint symptoms (1pt for > 6 weeks)

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15
Q

Diagnosis of SLE

A

Need 4/11 criteria:

Skin/mucosa (1 each) - rash (malar, discoid, or photosensitive), oral ulcers

Serosa (pleuritis or pericarditis)

Neuro disorder (seizures or psychosis)

Heme disorder (cytopenias/hemolytic anemia)

Kidney disorder (3+ protein, >500/24hr, cell casts)

Joint disorder (inflammatory polyarthritis)

Antibodies - 1 for ANA, 1 for other (dsDNA, a-Smith, APA)

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16
Q

Antibodies in RA

A

RF is 70% sensitive (50% initially, 60-80% later in established disease)

Anti-CCP is ~95% specific

Seronegative in 20%

Seronegative RA more likely in men

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17
Q

Antibodies in SLE

A

Antinuclear - 95% - Useful as an initial screening test

Anti–double-stranded DNA - 50% - Found in more severe disease, especially kidney disease; antibody levels commonly follow disease activity and are useful to monitor

Anti-Ro/SSA - 40% - Associated with photosensitive rashes, discoid lupus, and neonatal SLE, including congenital heart block; also common in Sjögren syndrome

Anti-U1-ribonucleoprotein - 35% - Associated with Raynaud phenomenon and esophageal dysmotility; also seen in MCTD

Anti-Smith - 25% - Specific for SLE; often associated with more severe disease

Anti-La/SSB - 15% - Common in Sjögren syndrome; less common in SLE and neonatal SLE

Antiribosomal P - 15% - Associated with CNS lupus and lupus hepatitis

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18
Q

What antibody combo definitively rules out SLE?

A

Negative ANA + negative Ro/SSA

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19
Q

In SLE, what is more useful - ESR or CRP?

A

ESR; some patients with SLE do not produce CRP in flares; if they do then CRP can be used however

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20
Q

Other than antibodies & ESR/CRP, what else should be measured in SLE?

A

Complements should also be assessed (most commonly, C3 and C4) because these levels are reduced during SLE activity

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21
Q

What are the classic drugs causing drug-induced lupus?

A

procainamide

methyldopa

quinidine

hydralazine

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22
Q

If a patient has a disease that looks similar to SLE but doesn’t meet full criteria, what would you call it?

A

Undifferentiated connective tissue disease

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23
Q

Labs that need serial monitoring in SLE:

A

CBC

ESR

anti-dsDNA

C3/C4

UA

UCr/Pr

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24
Q

Management of RA

A

DMARD monotherapy: low disease activity or moderate/high activity without poor prognostic features

DMARD combo therapy: Moderate or high activity with or without poor prognostic features

Biologics DMARDs: High activity with poor prognostic features Poor prognostic features: functional limitation, extra-articular disease, + RF or CCP, bony erosions

Non-biologics:

MTX is first choice, and the only one that can be used in combo with a biologic

Combo therapy can include HCQ +/- sulfasalazine

Leflunomide is an alternative if MTX is not a good option for one reason or another

Anti-TNF biologics:

anti-TNFs are the most commonly used (infliximab, adalimumab, etanercept, golimumab, certolizumab pegol)

Improvement is seen in weeks, more efficacious when used with MTX

Other biologics (use after one or two anti-TNFs have failed):

Abatacept (stops APCs presenting to T-cells; for severe disease)

Rituximab (depletes B cells; for severe disease)

Tocalizumab (anti IL-6)

Tofacitinib (JAK/STAT small molecule)

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25
Q

General management of SLE

A

Hydroxychloroquine for everyone (improves survival)

Prednisone 20-60/d for most acute manifestations

Prednisone 1000/d for most severe & life-threatening conditions

Mycophenolate mofetil for active lupus nephritis (more in nephrology section); Mycophenolate mofetil may be at least as effective as cyclophosphamide for systemic lupus erythematosus but with fewer, and milder, side effects

IV cyclophosphamide followed by mycophenolate (maybe azathioprine) for very severe conditions

Belimumab if all the above fail (add-on therapy)

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26
Q

The main types of scleroderma, and the main differences between them

A

Diffuse cutaneous: distal + proximal thickening, more often involving organ fibrosis & ILD, Raynaud’s usually accompanies onset of other symptoms

Limited cutaneous: Distal skin affected (face, neck, hands) without proximal (chest, abdomen, wrists up), typically no internal organ fibrosis however more likely to develop PAH, early Raynaud’s, and CREST

Morphea: localized plaques on the trunk, not associated with systemic manifestations

Linesar scleroderma: streaks/lines of thickened skin, not associated with systemic manifestations

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27
Q

Antibodies in systemic sclerosis

A

Anticentromere: LcSSc + PAH

Anti-scl-70 (DNA topoisomerase-1): DcSSc, ILD

Anti-RNA polymerase III: DcSSc, kidney disease

Anti-U3-RNP: DcSSc, PAH, myositis

Anti-PM-Scl: Myositis, associated with overlap polymyositis

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28
Q

Systemic sclerosis management

A

No “DMARD”; manage complications

Steroids should be used cautiously as they may precipitate a renal crisis

Cyclophosphamide for severe/rapidly progressive lung disease

ACEIs for mild HTN or unexplained creatinine elevations (prevent scleroderma renal crisis)

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29
Q

Treatment of Raynaud’s

A

avoid cold, avoid smoking

1st line: CCBs, anti-platelets, topical nitrates, aspiration

2nd line: PDE5i’s (sildenafil/tadalafil)

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30
Q

Systemic sclerosis clinical manifestations

A

Pulmonary (common): ILD, PAH, aspiration

Cardiac : cardiac fibrosis (rare but deadly), conduction disease (fibrosis), CAD

GI: Pharynx, esophageal, GERD, anal sphincter incompetence

Renal: Scleroderma renal crisis

Misc: Raynaud’s, Inflammatory arthritis, Peripheral neuropathy

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31
Q

Typical presentation & treatment of scleroderma renal crisis

A

acute kidney injury and severe hypertension, mild proteinuria, urinalysis with few cells or casts, microangiopathic hemolytic anemia, and thrombocytopenia. Some patients develop pulmonary edema and hypertensive encephalopathy. Occasionally, patients remain normotensive despite kidney dysfunction.

ACE inhibitors significantly improve kidney survival and decrease mortality among patients with SRC. Treatment with an ACE inhibitor should be initiated promptly in SSc patients with even mild hypertension or otherwise unexplained elevations in serum creatinine levels.

The ACE inhibitor should be up-titrated until good control of blood pressure is achieved and continued even in the setting of kidney disease.

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32
Q

Chronic steroid use necessitates consideration of what?

A

Vitamin D supplementation (all patients on chronic steroids or frequent tapers)

Bisphosphonate therapy (if >=5mg/d for > 4 weeks)

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33
Q

Adalimumab

A

TNF-alpha inhibitor

RA

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34
Q

Etanercept

A

TNF-alpha inhibitor

RA

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35
Q

Certolizumab pegol

A

TNF-alpha inhibitor

RA

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36
Q

Golimumab

A

TNF-alpha inhibitor

RA

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37
Q

Infliximab

A

TNF-alpha inhibitor

RA

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38
Q

Abatacept

A

T-call costimulation (APCs/Tcells)

RA

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39
Q

Rituximab

A

CD20 blocker (B-cells)

RA and ANCA vasculitis

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40
Q

Tocilizumab

A

IL-6 receptor

RA, JIA, Castleman disease

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41
Q

Belimumab

A

BLyS/BAFF

SLE

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42
Q

Tofacitinib

A

JAK

RA

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43
Q

Ustekinumab

A

IL-12/IL-23

Psoriasis; psoriatic arthritis

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44
Q

Anakinra

A

IL-1β receptor

RA, AOSD, cryopyrin-associated syndromes

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45
Q

Rilonacept

A

IL-1

Cryopyrin-associated syndromes, refractory gout

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46
Q

Canakinumab

A

IL-1β

Cryopyrin-associated syndromes

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47
Q

Methotrexate

A

DHFR inhibition

RA, psoriatic arthritis, DM & PM, vasculitis

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48
Q

Hydroxychloroquine

A

Unknown

SLE, RA

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49
Q

Sulfasalazine

A

Antimetabolite - a pro-drug broken down into 5-amino salicylic acid (active metabolite in GIT) and sulfapyridine (exerts systemic action)

RA, SpA, IBD

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50
Q

Leflunomide

A

Blocks dihydroorotase, enzyme involved in pyrimidine biosynthesis, antiproliferative

RA

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51
Q

Azathioprine

A

Purine analogue; inhibits DNA synthesis

SLE; DM; PM; vasculitis; IBD

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52
Q

Cyclophosphamide

A

Alkylating agent; blocks DNA synthesis

Severe and life-threatening disease in SLE, DM, PM, and vasculitis

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53
Q

Mycophenolate mofetil

A

Inosine monophosphate inhibition; antiproliferative; mycophenolate is converted into the active metabolite, mycophenolic acid, which inhibits inosine monophosphate dehydrogenase (an enzyme in the purine synthetic pathway) and preferentially inhibits T- and B-lymphocytes

SLE (especially lupus nephritis); vasculitis; DM; PM

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54
Q

General monitoring of DMARDs

A

Thereafter: CBC, LCTs, serum creatinine every 3-6 months

55
Q

Leflunomide monitoring

A

LCTs every 8-12 weeks, and leflunomide temporarily or permanently discontinued for significant elevations >2 times normal

56
Q

Hydroxychloroquine monitoring

A

Baseline/periodic ophthalmologic examinations approximately every 12 months to evaluate for hydroxychloroquine deposition, which rarely can lead to visual loss

57
Q

Cyclophosphamide monitoring

A

Urinalysis every 4-8 weeks

58
Q

Cyclosporine

A

Inhibits calcineurin (a transcription activating factor); preferentially targets T cells

SLE; psoriasis; RA

59
Q

Apremilast

A

Inhibits phosphodiesterase 4

Psoriasis; psoriatic arthritis

60
Q

Apremilast monitoring

A

Weight, neuropsychiatric effects

61
Q

General management of DMARDs

A

Key Points

◾Infection risk is elevated with most biologic agents; therapy should be temporarily interrupted during any significant infection.

◾Tumor necrosis factor α inhibitors are usually the treatment of first choice for patients with rheumatoid or psoriatic arthritis after inadequate response to nonbiologic disease-modifying antirheumatic drugs.

◾Other biologic agents are typically started after failure of one or two tumor necrosis factor α inhibitors, although some are also approved as first-line therapies.

62
Q

DMARDs and vaccines

A

Key Points

◾Whenever possible, patients should be brought up to date on vaccinations prior to starting immunosuppressive therapy.

◾Live attenuated vaccines are currently contraindicated for patients on biologic therapies; however, live attenuated vaccines may be administered approximately 4 weeks before starting biologic therapy.

◾Prior to initiating aggressive immunosuppressive therapy, screening for infections (tuberculosis, hepatitis B and C, HIV) is indicated; if needed, infection therapy must be started before initiating immunosuppressive therapy.

63
Q

Rheum medications and pregnancy

A

Key Points

◾Methotrexate is highly teratogenic and abortifacient and must be discontinued at least 3 months prior to conception.

◾Hydroxychloroquine is relatively safe in pregnancy and should not be discontinued.

◾Leflunomide is extremely teratogenic and must not be used before/during pregnancy; if leflunomide is inadvertently administered, cholestyramine treatment is required to remove the drug from the body before pregnancy.

64
Q

Radiographic findings of OA

A

Joint-space narrowing (articular cartilage loss)

Subchondral sclerosis

Marginal osteophyte formation

Subchondral cysts

65
Q

Erosive OA

A

OA with radiographic erosions seen Manifests clinically as intermittent flares of swelling and redness Differentiated from inflammatory arthritis due to central erosions (RA & psoriatic arthritis show marginal erosions) You should also see collapse of subchondral bone

66
Q

What is DISH?

A

Diffuse Idiopathic Skeletal Hyperostosis Essentially similar to OA of the spine, however classified differently Osteophytes of the anterolateral spine (often thoracic) on XRs, “bridging” of the osteophytes, and preservation of the disk spaces Radiographically, DISH may be difficult to differentiate from ankylosing spondylitis GI/airway complications: Dysphagia Aspiration pneumonia Difficult intubation Spine complications: Unstable spinal fractures Spinal stenosis Myelopathy

The prevalence of DISH increases with age and is approximately 15% in patients over 50 years of age

67
Q

Heberden & Bouchard nodes

A

Heberden = bony enlargement of DIP due to OA

Bouchard = bony enlargement of PIP due to OA

68
Q

When are steroid joint injections particularly useful?

A

One or limited number of joints

Effusions

69
Q

Criteria for diagnosis of fibromyalgia

A

High score on Widespread Pain Index (WPI) + Symptom Severity Scale (SSS)

WPI: self-reported pain for at least 3 months at 19 different body locations

SSS: severity of 3 symptoms: fatigue, waking unrefreshed, cognitive symptoms

ESR & CRP should be obtained and should usually be normal.

(The combination of a WPI of ≥7 plus an SSS of ≥5, or a WPI between 3 and 6 plus an SSS of ≥9, establishes a diagnosis of fibromyalgia for the purposes of study enrollment)

70
Q

Treatment for fibromyalgia

A

Exercise

pregabalin

SNRIs duloxetine & milnacipran

71
Q

Common features of spondyloarthropathies

A

inflammation of the axial skeleton, tendons, and entheses

tendon and enthesis calcification

association with HLA-B27

mucocutaneous, gastrointestinal, and ocular inflammation

72
Q

Unexpecedly severe cases of reactive or psoriatic arthritis should prompt suspicion of what?

A

HIV infection (+HLAB27)

73
Q

Presentation of ankylosing spondylitis

A

L-spine involvement that progresses upwards wtih time

Enthesitis (achilles?)

Psoriasis can coexist

Aortic valve disease; aortitis; conduction abnormalities; CAD

Restrictive lung disease from costovertebral rigidity; apical fibrosis (rare)

Falsely elevated bone mineral density from syndesmophytes; increased risk of spine fracture

Increased mortality (heart disease, cancer, infection)

74
Q

Presentation of psoriatic arthritis

A

Joint pattern:

  1. asymmetric lower extremity oligoarthritis

or

  1. symmetric polyarthritis of the hand joints (including DIP)

Psoriasis usually but not always precedes arthritis

Dactylitis & onycholysis relatively common

Spondylitis may occur but is usually asymmetric and skips regions (compare to ankylosing spondylitis)

RF/anti-CCP usually (~90+%) but not always negative

Radiographs: marginal joint erosions and new bone formation

75
Q

Presentation of IBD-associated inflammatory arthritis

A

Peripheral joint involvement may be:

oligoarticular (type 1)

or

polyarticular (type 2)

Only the oligoarticular peripheral arthritis parallels IBD activity

76
Q

Presentation of reactive arthritis

A

Occurs after specific gastrointestinal or genitourinary infections

Asymmetric monoarthritis or oligoarthritis in the lower extremities is the most common presentation

Enthesopathy (including at the Achilles tendon insertion to the calcaneus), dactylitis, and sacroiliitis may occur

Occurs approximately 3 to 6 weeks after the infectious trigger

Generally self-limited but can progress to a chronic disease

GI pathogens: Yersinia, Salmonella, Shigella, Campylobacter, and, rarely, Escherichia coli and Clostridium difficile

GU pathogens: Chlamydia trachomatis, Ureaplasma urealyticum

77
Q

Diagnostic evaluation of suspected spondyloarthritis

A
78
Q

How to diagnose which sponyloarthropathy it is

A
79
Q

Differentiation of DISH from ankylosing spondylitis on radiographs

A

the changes in ankylosing spondylitis are usually on both sides of the spine, whereas in DISH they are characteristically right sided

DISH is most commonly thoracic, whereas ankylosing spondylitis starts with sacroiliitis and lumbar arthritis and usually does not skip regions as it ascends

80
Q

Management of spondyloarthritis

A

Exercise (especially for ankylosing spondylosis)

Steroid injections (especially if a few joints)

NSAIDs (it can be helpful to try a second NSAID if the first has produced an inadequate response)

For ankylosing spondylitis & psoriatic arthritis: go straight to anti-TNFs

For psoriatic arthritis: Ustekinumab has shown to be really good, can be used with MTX

For IBD-associated & reactive: try non-biologic DMARDs before anti-TNFs

In general, non-biologics do not help with axial disease

81
Q

What is sjogren syndrome?

A

immune-mediated disease of unknown cause

manifests as infiltrative inflammation that damages exocrine glands, including:

major and minor salivary glands

lacrimal glands

less commonly, other exocrine glands such as the pancreas

82
Q

Clinical manifestations of sjogren syndrome

A

sicca, or dryness, particularly of the:

eyes (keratoconjunctivitis sicca; result in corneal damage and visual impairment)

and

mouth (xerostomia; can result in dental caries)

Can have extra-glandular manifestations

Most common = fatigue and arthralgia

Can affect almost any organ it looks like

increased risk of lymphoma, with diffuse large B-cell and mucosa-associated lymphoid tissue (MALT) lymphomas being the most common

83
Q

Schirmer test

A

Documenting reduced tear production (decreased wetting of tear test strips)

Used for diagnosis of Sjogren’s & lacrimal gland dysfunction

84
Q

Diagnosis of Sjogren’s

A

Clinical presentation

Laboratory findings include positive autoimmune serologies (rheumatoid factor, antinuclear, anti-Ro/SSA, and anti-La/SSB antibodies)

Hypergammaglobulinemia

Anti-Ro/SSA and anti-La/SSB antibodies are characteristic for Sjögren syndrome

Gland biopsy (usually lip) is considered the gold standard

Other autoimmune diseases, including rheumatoid arthritis, systemic lupus erythematosus, and autoimmune thyroiditis, are commonly associated with Sjögren syndrome.

85
Q

Treatment of Sjogren’s

A

Topical/symptomatic (artifical tears, lubrication)

NSAIDs and steroids

Non-biologic DMARDs

86
Q

Definition of MCTD

A

Mixed connective tissue disease

Overlap syndrome that includes features of:

SLE

SSc

And/or polymyositis

In the presence of:

anti-U1-ribonucleoprotein (RNP) antibodies

87
Q

Diagnosis of MCTD

A

Numerous clinical manifestations; any features of SLE, SSc, or PM

Very high ANA titer (≥1:1200) in a speckled pattern

Positive anti-U1-RNP antibodies; presence of anti-Smith or anti–double-stranded DNA antibodies should suggest SLE rather than MCTD

88
Q

Pathophysiology of gout

A

Purines are created by cell turnover, are converted to xanthine/hypoxanthine, then converted by xanthine oxidase to uric acid

In purine overproduction, overconsumption, or under-excretion, macrphages eat the crystals and release cytokines causing neutrophil infiltration and an inflammatory cascade

89
Q

Synovial fluid analysis of crystal arthropathy

A

monosodium urate crystals are needle shaped and negatively birefringent

CPP crystals are rhomboid shaped and positively birefringent

gout fluid is typically inflammatory (2000/µL to >100,000/µL [2.0-100 × 109/L], neutrophil predominance of ≥50%)

Whereas extracellular crystals confirm a chronic gout diagnosis, crystals within neutrophils define active, gout-induced inflammation

Gram stain and cultures must be obtained to exclude infection. Acute gout and joint infection occasionally coexist.

90
Q

Raiographic findings of gout

A

Normal in acute/short-duration gout

In long-standing uncontrolled disease:

punched-out lesions

often with overhanging edges where the bones have been eaten away by tophaceous deposits

91
Q

Treatment of acute gouty arthritis

A

Colchicine

NSAIDs

Steroids

Intra-articular steroids are very effective

Patients already taking urate-lowering therapy should continue at the same dose throughout a flare because changes in serum urate can exacerbate attacks

92
Q

Foods that elevated purine levels

A

Meat

Beer

High-fructose (cokes, processed foods)

93
Q

Urate lowering therapy in gout

A

ACR vs EULAR

Urate-lowering therapy

Flare prophylaxis

ACR: “treat to avoid symptoms”

EULAR: “treat to target”

< 6.0 wtihout tophi

< 5.0 wtih tophi

Both the ACR and EULAR recommend urate-lowering therapy for patients with gout plus any of the following: (1) ≥stage 2 CKD; (2) ≥2 acute attacks per year; (3) one or more tophi; or (4) uric acid nephrolithiasis.

1st line = usually allopurinol, febuxostat is a more expensive but maybe more efficacious option

2nd line = probenecid (however may increase risk of renal stones and doesn’t work as well in CKD)

3rd line = the potent intravenous agent pegloticase is a treatment option; provides uricase activity, converting uric acid to allantoin

Start flare prophylaxis when starting urate-lowering therapy (colchicine & NSAIDs preferred, steroids 2nd line)

ACR currently recommends that prophylaxis should be continued for the greater of the following: 6 months; 3 months after achieving the target serum urate level for a patient without tophi; or 6 months after achieving the target serum urate level where there has been resolution of tophi

94
Q

The four clinical presentations of CPPD & their treatments

A

Calcium Pyro-Phosphate Deposition

  1. cartilage calcification (also known as chondrocalcinosis)

tx = no specific tx

  1. acute CPP crystal arthritis (also known as pseudogout)

tx = NSAIDs, colchicine, steroids

  1. chronic CPP crystal inflammatory arthritis (“pseudo-rheumatoid arthritis”)

tx = NSAIDs, colchicine, steroids, MTX, HCQ

  1. osteoarthritis with CPPD (accelerated osteoarthritis findings in joints not commonly involved with simple osteoarthritis)

tx = as regular OA

95
Q

The diseases associated with CPPD are:

A

hyperparathyroidism

hemochromatosis

hypomagnesemia

hypothyroidism

previous joint injury

96
Q

What is “Basic Calcium Phosphate Deposition”

A

common cause of cartilage calcification and may be radiographically indistinguishable from CPPD

Unlike CPP crystals, BCP crystals also commonly deposit in periarticular tendons, bursae, and other soft tissues

Patients are often asymptomatic, but BCP crystals can cause destructive arthropathy

BCP crystals also can stimulate inflammation via pathways similar to monosodium urate and CPP crystals

Diagnosis of BCP deposition is made clinically or by joint aspiration

Synovial fluid is characteristically noninflammatory (<2000/µL)

BCP crystals are not visible on polarized microscopy

Treatment for symptomatic BCP deposition includes NSAIDs, joint aspiration and tidal lavage, and intra-articular glucocorticoid injection

97
Q

Synovial fluid analysis of infectious arthritis

A

leukocyte count is markedly increased (usually >50,000/µL [50 × 109/L] with neutrophil predominance)

sensitivity of cultures are only 80% and may be negative due to precefing antibiotics, so clinical assessment ultimately guides treatment

98
Q

Presentation of gonooccal arthritis

A

Actually has two main presentations:

  1. Grossly purulent mono- or oligoarthritis
  2. Dissemminated infection with rash, constitutional symptoms, and polyarthralgia, and tenosynovitis of the hand and feet; leukocyte counts are lower (<25k)
99
Q

Presentation of Lyme arthritis

A

Although arthralgia is common in early Lyme disease, Lyme arthritis generally refers to frank inflammation of the joints as a manifestation of late Lyme disease

Impressively large effusions, most often a knee monoarthritis with prominent stiffness but relatively little pain

Diagnosis is made by serologic testing (Borrelia burgdorferi)

Joint aspiration demonstrates a moderately inflammatory synovial fluid (leukocyte count typically 20,000-25,000/µL [20-25 × 109/L], neutrophil predominant)

100
Q

MTB arthritis

(mycobacterium tuberculosis)

A

most commonly presents as spondylitis (Pott disease) or vertebral osteomyelitis

chronic, indolent course, often without constitutional symptoms

monoarticular, often involving the hip or knee

Synovial fluid analysis usually suggests a nonspecific inflammatory process

101
Q

Mycobacterium marinum infections

A

typically begin as red or violaceous plaques, nodules, or abscesses in the skin, and may spread locally to involve the hand joints

102
Q

Viral infections causing arthritis

A

HBV:

symmetric polyarthritis, especially of hands & knees, often with rash

HCV:

chronic arthirits, numerous presentations

Parvo B19:

usually gotten from an infected child, symmetric arthritis with swelling of small joints (hands & feet, wrists & knees), lasting weeks to months, dx = IgM (not IgG)

Rubella:

Small joint polyarthritis

Associated with rash, fever, and lymphadenopathy

Resolves in ~2 weeks

dx = IgM

Can occur in response to the vaccine

Chikungunya:

From asia & africa, mosquito virus

fever, rash, myalgia, thrombocytopenia and/or leukopenia

Self-limited polyarthritis/tenosynovitis but lasting up to 6 months

IgM

103
Q

Early vs delayed vs late prosthetic joint infections

A

Early is < 3 months

Acute pain and swelling

Acquired during prosthesis implantation

Delayed is < 12 months

Prolonged joint pain, often without fever

Acquired during prosthesis implantation

Late is > 12 months

Acute pain and swelling

De novo from hemtogenous seeding

104
Q

Describe the three idiopathic inflammatory myopathies and their proposed pathophysiologies

A

PM (polymyositis) is a CD8-positive T-cell–mediated immune disease with direct myocyte injury

DM (dermatomyositis) is considered an immune complex disease with vascular inflammation in muscle and subsequent muscle damage

IBM (inclusion body myositis) is most likely a myodegenerative disorder with vacuolar inclusions and a related T-cell response

105
Q

What is antisynthetase syndrome?

A

Seen in DM and PM

Defined by the presence of autoantibodies to aminoacyl-transfer (t)RNA synthetase enzymes (such as anti–Jo-1), plus two of the following clinical features:

inflammatory myositis

interstitial lung disease

Raynaud phenomenon

nonerosive inflammatory arthritis

“mechanic’s hands”

106
Q

Muscle involvement in the idiopathic inflammatory myositis syndromes

A

Symmetric painless proximal weakness of the arms and legs is the classic feature of DM and PM:

difficulty combing hair, rising from a chair, and climbing stairs (classic triad of hair, chair, stair)

difficulty with routine activities

muscle weakness in IBM characteristically affects both distal and proximal muscles

Onset is insidious and slowly progressive, often over years

107
Q

Cutaneous involvement in dermatomyositis

A

Gottron sign/papules:

symmetric erythematous/violaceous macules, patches, or papules located on the extensor surfaces of the metacarpophalangeal joints

heliotrope rash:

edematous lilac discoloration of periorbital tissue

photodistributed rashes:

shawl sign (upper back)

V sign (neck/upper chest)

Poikiloderma:

mottled pigmentation, epidermal atrophy, and telangiectasia in both sun-exposed and unexposed areas

Gottron sign/papules and heliotrope rash are considered pathognomonic for DM

108
Q

Amyopathic dermatomyosits

A

classic cutaneous findings of dermatomyositis occurring in the absence of muscle involvement

109
Q

Main cardiopulmonary involvement in PM/DM

A

ILD:

Often associated with antisynthetase antibodies, including anti–Jo-1

Poor prognostic finding

Routine screening for ILD with imaging such as chest radiography or CT is appropriate in asymptomatic patients in the presence of antisynthetase antibodies

Diaphragm muscle weakness

resulting in shortness of breath and, occasionally, respiratory failure

Pharyngeal muscle involvement

aspiration pneumonia

110
Q

How to differentiate PM/DM from IBM

A

DM/PM:

Female, younger, ass’n with cancer

Proximal only

CK/aldolase > 10x ULN

Acute/subacut onset & rapidly progressive

Responds to therapy

IBM:

Male, older (>50), familial ass’n

Proximal + distal

CK < 10x ULN

Insidious onset & slowly progressive ( large impact on daily activities but little impact on overall survival)

Does not respond to therapy

111
Q

Antibodies in idopathic inflammatory myositis

A

DM/PM:

ANA (80%)

Myositis-specific autoantibodies (MSA): checked 1st

anti–Jo-1

antibodies to signal recognition particle (SRP)

antibodies to Mi-2

Myositis-associated autoantibodies (MAA): checked 2nd

anti-PM-Scl

anti-Ku

anti-Ro/SSA

anti-La/SSB

anti-U1-ribonucleoprotein (RNP) antibodies

Positive anti-U1-RNP antibodies suggest an overlap syndrome with mixed connective tissue disease.

No Abs for IBM

112
Q

Ddx of idiopathic inflammatory myopathies

A

hereditary diseases

infection

endocrine

drug-induced myopathies (colchicine, hydroxychloroquine, steroids)

113
Q

Treatment of idiopathic inflammatory myopathies

A

Steroids are the mainstay of therapy

Immunosuppressive therapy with methotrexate or azathioprine is used for glucocorticoid-resistant disease or ILD

mycophenolate mofetil and leflunomide

IVIG is recommended as an alternative treatment for DM

114
Q

Giant cell arteritis:

  1. Clinical findings
  2. Affected arteries
  3. Management
A
  1. Clinical findings:

headache

jaw claudication

amaurosis fugax & blindness

decreased pulses and hand or forearm ischemia

aortic aneurysm and aortic dissection

elevated ESR

age > 50

  1. Affected arteries:

external carotids

temporal arteries (hence the alternative designation temporal arteritis)

ciliary and ophthalmic arteries

subclavian and brachial arteries can be affected

  1. Management:

Treat with steroids empirically, total of 6-18 months

Biopsy may miss the affected area anyways (not super sensitive)

Still can interpret biopsy even after up to 2 weeks after treatment initiation

ESR can serve as a marker of disease activity

115
Q

Polymyalgia Rheumatica

  1. Diagnosis
  2. Treatment
A
  1. Diagnosis:

clinical diagnosis based on the characteristic symptoms in a patient older than 50 years

pain & stiffness in the shoulder, neck, and hips

inability to comb hair or rise from a chair unassisted

creatine kinase and aldolase are generally normal

elevated ESR

  1. Treatment:

low-dose prednisone, good response (but often relapses wtih steroid discontinuation so may be on steroids for a long time)

116
Q

Takayasu Arteritis

  1. Affected arteries
  2. Clinical findings
  3. Management
A
  1. Affected arteries:

aorta (any part) and its major branches.

  1. Clinical findings:

young women, typical age at onset between 15 and 25 years

fever, fatigue, malaise, weight loss, arthralgia and myalgia often precede the onset

narrowings & dilations of large arteries

diminished or absent pulses often accompanied by bruits

asymmetric BP measurements

hypertension (aortic coarctation)

AR (aortic root aneurysm/dilation)

  1. Management:

High-dose prednisone (1mg/kg/d) then taper

117
Q

Polyarteritis Nodosa

  1. Vessels involved
  2. Clinical findings
  3. Management
A
  1. Vessels involved:

Aneurysms and microaneurysms of medium & small arteries

kidneys, gastrointestinal tract (especially the mesenteric artery and small intestine), peripheral nervous system, and skin

Coronary arteries can also be affected

  1. Clinical findings:

fatigue, malaise, fever, myalgia, arthralgia

hypertension (renal artery involvement; not GN)

abdominal angina (mesenteric artery invovlement) & bowel infarction

mononeuritis multiplex (peripheral nervous system invovlement)

skin involvement (livedo reticularis, purpura, and painful subcutaneous nodules)

hepatitis B association (30%)

  1. Management:

Can have a poor prognosis

Treatment is aggressive, including high-dose prednisone and cyclophosphamide

Patients with hepatitis B should receive plasma exchange and antiviral therapy whenever feasible

118
Q

PACNS

A

“Primary Angiitis of the Central Nervous System”

Necrotizing granulomatous vasculitis limited to the vessels of the CNS

Recurrent headaches with progressive encephalopathy

Can also see strokes, seizures, and visual field deficits

Difinitive test is brain biopsy showing granulomatous vasculitis

Management is aggressive and includes high-dose glucocorticoids and daily oral cyclophosphamide

But generally is unstoppable and leads to cognitive decline, dementia, and death

119
Q

Granulomatosis with polyangiitis

  1. Clinical findings
  2. Management
A
  1. Clinical findings:

“C-disease”

upper respiratory tract (including sinuses and ears), lungs, and kidneys

c-ANCA pattern and anti-PR3 antibody positivity

  1. Management:

Treatment can be started with c-ANCA and clear presentation

Biopsy may be helpful

Kidney biopsies will generally not show granulomas however so may mkae it difficult to distinguish from other ANCA diseases

Glucocorticoids alone are insufficient to control GPA:

Treatment includes high-dose glucocorticoids plus cyclophosphamide or rituximab

Followed by maintenance therapy with methotrexate, azathioprine, mycophenolate mofetil or rituximab for at least 24 months

120
Q

Microscopic polyangiitis

  1. Clinical findings
  2. Management
A
  1. Clinical findings

kidneys & lungs

kidneys = almost 100%, and as in GPA, the lesions are those of necrotizing glomerulonephritis; immune deposits are sparse or absent

lungs = non-granulomatous alveolar infiltrates, may have DAH which can be fatal

p-ANCA and anti-MPO antibodies

  1. Management:

high-dose glucocorticoids plus either cyclophosphamide or rituximab

followed by maintenance therapy with methotrexate, azathioprine, or mycophenolate mofetil.

121
Q

Eosinophilic Granulomatosis with Polyangiitis

A
  1. Clinical findings:

history of atopy, including allergic rhinitis, nasal polyps, or asthma

Hypereosinophilia = both the peripheral blood and involved tissues; diagnosis of EGPA should be revisited in the absence of eosinophils

Lung disease = infiltrates and capillaritis

Peripheral nerve disease = mono- or polyneuropathy or mononeuritis multiplex

Kidney disease = less common than in other ANCA diseases

typically p-ANCA/anti-MPO pattern however may be ANC negative

  1. Management:

mild or limited disease = glucocorticoid treatment alone may therefore be sufficient

severe disease = glucocorticoids plus cyclophosphamide is preferred, followed by maintenance therapy with a less toxic immunosuppressive agent

122
Q

Small vessel vasculidities: ANCA vs immune complex

A

ANCA = c- vs p-, “pauci-immune”

GPA

MPA

EGPA

Immune-complex = “leukocytoclastic vasculitis”

Cryoglobulinemic Vasculitis

Henoch-Schönlein Purpura

Hypersensitivity Vasculitis

123
Q

Cryoglobulinemic Vasculitis

  1. Clinical findings
  2. Associations
  3. Management
A
  1. Clinical findings:

precipitating in vitro at temperatures below normal body temperature

palpable purpura + other organ (usually glomerulonephritis + peripheral nerves, sometimes other organs)

cold-related precipitation, vascular ischemia, and infarction of the fingertips, ear helices, and tip of the nose

  1. Associations:

Hematologic malignancy = monoclonal IgM

HCV = Polyclonal IgG + monoclonal IgM + RF

Autoimmune disease (SLE/RA) = Polyclonal IgG + polyclonal IgM + RF

  1. Management:

Mild disease = treat underlying cause

Severe disease = steroids, cyclophosphamide/rituximab, plasmapharesis

124
Q

Henoch-Schönlein Purpura

A
  1. Clinical features:

IgA depositions

Palpable purpura (palms and soles)

Abdominal pain wtih GI ischemia & bleeding

Glomerulonephritis (IgA nephropathy)

  1. Management:

Steroids

125
Q

Hypersensitivity Vasculitis

A
  1. Clinical findings

Response to known or unknown antigen (ie drug or infection)

Immune-complex disease, usually not IgA

Palpable purpura and other rashes

Other organs are usually spared

  1. Management:

If mild/moderate = remove offending antigen and rash should resolve within weeks

If severe = steroids

126
Q

Behçet Syndrome

A

a form of vasculitis that affects small to large arterial vessels and can affect veins as well

Diagnosis:

Recurrent painful ulcertain of mouth or tongue

+

One other manifestation:

Eye involvement (distinctive lesion is Hypopyon; a layered collection of pus in the anterior chamber)

Skin involvement (Erythema nodosum, acneiform lesions especially of the thighs or trunk)

Pathergy test (transient subcutaneous insertion of a sterile, large-bore needle under the skin results in the development of a pustule at the site 24-48 hours later)

Genetics:

HLA-B51

Other manifestions:

pulmonary artery, aorta, and/or femoral artery inflammation (3%-12% of patients). Aneurysms and stenosis can occu

headaches, stroke, pyramidal signs, behavioral changes, or, rarely, dural sinus thrombosis

Gastrointestinal involvement may be hard to distinguish from inflammatory bowel disease but ulceration typically involves the ileum rather than the rectal or perianal regions and does not lead to fistula formation

Treatment:

Low-dose prednisone or colchicine is used for oral/genital ulcers

high-dose prednisone and immunomodulating agents such as azathioprine are used for more severe disease

Tumor necrosis factor (TNF)-α inhibitors, interferon alfa, and anti-interleukin (IL)-1β therapy have been used in recalcitrant or severe cases.

127
Q

Relapsing Polychondritis

A

autoimmune response to type II collagen

Need 3/6 of the next two categories:

Chondritis of:

Ears (helix, not lobes) (recurrent)

Nose (“saddle nose” deformity)

Larynx or trachea (airway stenosis)

Also:

Nonerosive inflammtaory polyarthiritis

Eye inflammation

Chochlear/vestibular dysfunction

Associations:

Age 60s70s –> look for MDS

Large vessel vasculitis

Other autoimmune diseases

Treatment:

Low dose prednisone for mild disease

High dose prednisone for life-threatening disease

128
Q

Adult-onset Still Disease

A

high spiking fevers

a salmon-colored rash

arthritis

high neutrophil counts

macrophage hyperactivation

can have severe and life-threatening dysfunction of almost any organ

diagnosis of exclusion (exclude infection, malignancy, other rheum disease)

ferritin is generally very high

NSAIDs and/or prednisone

129
Q

Familial Mediteranean Fever

A

MEFV1 gene

Attacks every 1-3 days:

Polyserositis

Arthritis

Erysipeloid rash around ankles

Elevation of acute-phase reactants

AA amyloidosis is a potential long-term consequence

Treatment = colchicine, chronic daily treatment

130
Q

Löfgren syndrome

A

specific syndrome of sarcoidosis

younger adults

acute “arthritis”

(actually a nondestructive periarthritis of the soft tissue, entheses, and tenosynovium)

bilateral hilar lymphadenopathy

erythema nodosum

131
Q

Genes associated with the true connective tissue diseases

Marfans

Ehlers-Danlos

Osteogenesis Imperfecta

A

Marfans

fibrillin 1 (FBN1)

Ehlers-Danlos

collagen-related genes (COL)

or the procollagen lysyl hydroxylase(PLOD)

Osteogenesis Imperfecta

COl genes (type I collagen)

132
Q

Osteogenesis imperfects

A

Blue sclera

Broken bones

Bisphosphonates

COL gene (collagen type I)

133
Q

Marfans vs Ehlers-Danlos

A

Marfans:

tall

arachnodactyly

anterior thoracic deformity

spinal curvature

sking & ocular involvement

aortopathy & MVP common

Echo at the time of diagnosis & at 6 months, then yearly if stable

Threshold is 5.0 usually

E-D:

joint hypermobility

stretchy skin

atrophic scars

velvety skin

“periodic” echo to assess root size

134
Q

IgG-4 related disease

A

abundant IgG4-producing plasma cells

Almost any organ can be involved; lymph nodes are frequently affected

Diagnosis is made by tissue biopsy and the demonstration of a characteristic histology

(lymphs & plasmas with IgG-4 and no neutrophils/granulomas)

*** Serum levels are not always elevated!!! ****

Treatment = steroids

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