Arthritis, Connective Tissue Disease and Vasculitis Flashcards

1
Q

Joints affected by Inflammatory arthritis (RA)

A

Small Joints, sparing the DIP

PIP, MCP, MTP, wrist

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2
Q

Pain pattern of an inflammatory arthritis (RA)

A

Improves with activity
Worse in the morning
>30 minutes of morning stiffness

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3
Q

Joints affected by Non-inflammatory arthritis (OA)

A

Large joints, and DIP

Knee, hip, shoulder, spine

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4
Q

Synovial fluid appearance in inflammatory arthritis

A

Cloudy, non-viscous (only non-infectious synovial is straw colored and clear)

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5
Q

Diseases that cause a cloudy, non-viscous synovial aspirate

A

RA, spondylitis, crystalline arthritis, viral/fungal infection
(inflammatory arthritis)

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6
Q

Joints most commonly affected by gout

A

Large toe
Knee
Ankle
Midfoot

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7
Q

Precipitating events for gout flares (9)

A
Alcohol
Infection
Surgery
CKD
Diuretics
Aspirin
Obesity
High protein meals
Cyclosporine
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8
Q

Main characteristics of an inflammatory arthritis

A

Stiffness >30 minutes
Morning Pain
Better with activity
Affects small joints most

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9
Q

Symptoms of an acute gout flare

A

Pain, swelling, and warmth in affected joint
Fever or sweating
Leukocytosis

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10
Q

Tophi

A

Deposits of urate crystals within the body

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11
Q

Locations that tophi can occur in gout patients

A

Fingers
Wrists
Knees
Olecranon
Pressure points (ulnar forearm, achilles tendon)
Visceral locations (heart, kidney, sclera)

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12
Q

Birefingence of gout crystals

A

Negative (gout = doubt)

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13
Q

Birefringence of CCPA crystals

A

Positive

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14
Q

Radiographic findings of gout joints

A

Erosive arthritis: punched out joints with overhanging bone lesions

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15
Q

Mechanism of damage in CCPA (calcium pyrophosphate arthritis)

A

Neutrophils swallow crystals and cause inflammation

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16
Q

Drugs of choice for managing an acute gout flare

A

Colchicine
NSAIDs
Steroids

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17
Q

Primary symptoms of CCPA

A

Mimics gout in pain and presentation
Does not have tophi typically, but DOES have dactylitis AKA boxer glove swelling
(also seen in RA and psoriatic arthritis)

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18
Q

Other diseases associated with CCPA

A

Hyperparathyroidism (high levels of Ca –> CPP deposition)
Hypothyroidism
Hemochromatosis
Hypomagnesemia (Calcium absorbed preferentially due to low levels)
hypophosphatemia (consumed in CPP crystals)

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19
Q

Factors that can precipitate CCPA/Pseudogout

A

Surgery
Infection
Trauma

But NOT alcohol like GOUT

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20
Q

Joints most commonly affected by CCPA

A

Knee
Wrist
Hand
Ankles

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21
Q

Treatment of choice for an acute CCPA flare

A

Same as gout
NSAIDs
Steroids
Cholicine

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22
Q

Prognosis of pseudogout

A

Typically resolves within 5-14 days

Chronic CPPA increases risk of osteoarthritis

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23
Q

MoA of Colchicine

A

Arrests microtubule assembly and inhibits inflammatory cells (via inhibition of cell division)

But only effective if given within 48 hours of a flare

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24
Q

Precautions for Colchicine Use

A

Affects Highly dividing cells: GI upset, bone marrow suppression.
Myopathy

CI in kidney disease due to electrolyte imbalances

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25
Q

NSAID of choice in treating gout

A

Indomethacin

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26
Q

Contraindications of indomethacin use in acute gout flares

A
Renal insufficiency (NSAIDs can precipitate renal hypoxia)
Gastric ulcers
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27
Q

Treatment of choice for gout flares in patients with kidney disease or polyarticular manifestation

A

Steroids (colchicine and indomethacin are CI in kidney disease)

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28
Q

Drugs commonly used to treat chronic gout

A

Allopurinol (w/ colchicine)
Febuxostat
Uricase (uricosuric)
Probenecid

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29
Q

MoA of febuxostat

A

Xanthine oxidase inhibitor similar to allopurinol but with low risk of hypersensitivity reaction

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30
Q

Criteria for chronic gout arthritis

A

3+ flares in a year or 5+ lifetime flares
Urate level >13
Uric acid nephropathy
Tophi or erosions on radiograph
Prophylaxis to prevent tumor lysis syndrome when starting chemotherapy

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31
Q

Precautions in allopurinol use

A

Potential for hypersensitivity/SJS reaction (do not rechallenge)
Can worsen flares (give with colchicine)
Fever
Cytopenia (risk increased with azathioprine for RA or organ rejection)

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32
Q

Probenecid MoA

A

Increases uric acid excretion (requires >700mg/day clearance to be effective)

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33
Q

Uricase contraindications

A

Anaphylaxis to IV infusion

Cost

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34
Q

Situations in which uricase would be used for gout

A

Severe tophaceous or refractory gout due to its price

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35
Q

“Shared epitope” that increases RA risk in smokers

A

HLA-DRB1

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36
Q

HLA isotypes associated with RA

A

HLA-DR 1, 4, 10

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37
Q

Serum markers for RA

A

Rheumatoid factor
Anti-citrullinated protein antibody
Elevated ESR and CRP

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38
Q

Polyarthritis options if the patient does not have synovitis (non-inflammatory polyarthritis)

A
Osteoarthritis
Viral arthralgia (hepatitis especially)
Mechanical damage
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39
Q

Characteristic symptoms of rheumatoid arthritis

A
Synovitis
Pain lasting >6 weeks
Pain is most often symmetric and proximal
Joint erosion
Extra-articular manifestations
40
Q

Diseases in which rheumatoid factor may be positive

A
Rheumatoid arthritis
Sjogren's syndrome
sarcoid
endocarditis
hepatitis C infection (or hepatitis C arthritis)
cryoglobulinemia
41
Q

Diseaes in which anti-citrullinated protein antibodies may be positiveq

A

RA only, much more specific than RF and can predate symptoms by a few years

42
Q

Characteristic radiographic findings for RA

A
Pannus formation within the joint space
marginal erosions
Symmetric joint space narrowing
Ulnar deviation of joints
Boutinniere joint (DIP extension)
Swan neck (DIP flexion)
Marginal erosions of bone near the synovium
Rheumatic nodules
43
Q

Extra-articular manifestations of RA

A

Skin: Nodules, scleritis, nail fold infarction, vasculitis ulcers
Lung: ILD, pleurisy, pulmonary nodules
Cardiac: accelerated CVD, myocardial nodules, aortitis (vasculitis)
Felty’s Disease: Anemia, thrombocytopenia, neutropenia, splenomegaly
Rheumatoid vasculitis

44
Q

Drugs of choice for RA

A

Immunosuppression
Anti-inflammatories
DMARDs (hydroxychloroquine, methotrexate, lefunamide, sulfasalazine)
Anti-TNF agents

45
Q

“Triple Therapy” of RA

A

Methotrexate + Sulfasalazine + Hydroxyquinone

= Anti TNFa effectivness

46
Q

MoA for hydroxychloroquine in mild RA and Lupus

A

Reduces lysosomal fusion
Reduces DNA synthesis
Reduces platelet aggregation
Reduces cytokine release

47
Q

Toxicity associated with hydroxychloroquine

A
Retinal or corneal deposits
Vertigo
Hypersensitivity reactions
Neuropathy
Cardiomyopathy
48
Q

Toxicities associated with Methotrexate (non-obvious)

A

Acute or chronic pulmonary or liver toxicity
Major teratogen
CI in alcoholics

49
Q

Mechanism of Leflunomide in RA treatment

A

Similar to methotrexate but affects dihydrooate and not foalte metabolism

50
Q

Toxicities of Leflunomide in RA treatment

A
GI upset
Alopecia
HTN
cytopenia
liver toxicity (CI in alcoholics)
teratogen
(all similar to methotrexate except hypertension)

Can be easily reversed by cholestyramine in the case of an acute infection

51
Q

Toxicities associated with sulfasalazine in RA treatment

A

GI upset, headache
Aplastic anemia, hepatitis, nephritis, pancreatitis are all rare.

Cannot be used with sulfa allergy
CAN be used in alcoholics

52
Q

Review the biologic agents for RA

A

Review the biologic agents for RA

53
Q

Primary concern in treating RA with rituxumab

A

Risk of hepatitis B reactivation or progressive multifocal leukoencephalopathy

54
Q

Primary concern of anti-TNFa biologics

A

formation of anti-dsDNA and Lupus drug reaction

55
Q

Unique features of Systemic Onset JIA

A

Least common JIA
Presents with fever, rash, and nodules (systemic part)
Affects any joint
Destructive arthritis, leukocytosis, anemia, and ESR are all marked
ANA is absent

56
Q

Unique features of Pauciarticular JIA

A

Most common JIA but hard to diagnose due to relative lack of symptoms (Pauci)
Occurs early in childhood and is rare over age 10
Affects large joints but spares the hip (other JIAs will affect hip)
Uveitis
+ANA

57
Q

Unique features of polyarticular JIA

A

Affects any joint, but the hip is never first (vs pauciarticular, which always spares hip)
Has less destructive arthritis, anemia, and ESR than systemic JIA
Only JIA that can possibly have a +RF

58
Q

HLA isotype associated with ankylosing spondylitis and reactive arthritis

A

HLA-B27 (explains male gender preference in AS and reactive arthritis)

59
Q

Shober manuver

A

Reduced back flexibility indicative of splondyloarthropathy

60
Q

Common features of spolyloarthropathies

A

Very responsive to NSAIDs
Dactylitis (some)
Uveitis (ALL)
Strong association with HLA-B27

61
Q

Features of Psoriatic Arthritis

A

Low Back Pain
Psoriasis
Asymmetric distribution of spondylitis
Dactylitis (sausage fingers)

62
Q

Features of Aknylosing Spondylitis

A
Higher incidence in males
High corellation with HLAB27
Symmetric sacroilitis
Enthesitis (not seen only in IBD)
Anterior uveitis
Prostatitis (unique)
63
Q

Features of Reactive Arthritis

A
Male predominancce
Often occurs after an STI or GI infection
Anterior uveitis, conjunctivitis
Commonly affects large joints
High HLA-B27 assocaiation
SKIN LESIONS: Circinate balatitis, keratoderma
Urethritis
Oncholysis

Can’t see, can’t pee, can’t bend my knee

64
Q

Symmetric spondylopathies (2)

A

AS and Reactive

65
Q

Asymmetric spondylopathies (2)

A

Psoriatic and IBD associated

66
Q

Only spondyloarthritis without enthesis

A

IBD

67
Q

Therapeutic options for ankylosing spondyltitis

A

ROM excercises to avoid spinal fusion by syndesmophytes
NSAIDs
Sulfasalazine or methotrexate
TNF inhibitors

68
Q

Presenting Symptoms of Lupus

A
SOAP BRAIN MD
Serositis
Oral and nasal ulcers (PAINLESS)
Arthritis
Photosensitivity

Blood dyscrasias (anemia, thrombocytopenia, hemolysis is Coombs positive)
Renal disease
Autoimmune studies (+ANA)
Immunologic (anti-smith, anti-dsDNA, anti-PL)
Neurologic (seizures, psychosis)

Malar rash
Discoid rash

Also libman sacks endocarditis (vegetations on valves)

69
Q

Number of SOAP BRAIN MD symptoms needed to Dx Lupus

A

4/11 of these symptoms are needed to diagnose Lupus

70
Q

Risk factors for developing Lupus

A

Genetic factors on X chromosome (all females, males with Kleinfelter)
Estrogen therapy or early menarche
Childhood EBV infection
Exposures to smoking, silica, dust, UV light

OOPHORECTOMY can alleviate some risk in females

71
Q

Primary mechanism of kidney disease in Lupus

A

Type III: immune complex deposition

72
Q

Antibodies positive in Lupus

A

ANA (non-specific, highly sensitive)
Anti-dsDNA
Anti-phospholipid
Anti-smith
Anti-cardiolipin (causes cross reactions in VDRL and falsely prolongs PTT)
Anti-histone (in drug-induced lupus only)
Hypocomplementemia

73
Q

Treatment for Lupus

A

NSAIDs steroids
Hydroxychloroquine
Cyclophosphalmide/mycophenolate only if severe disease
Rituxumab

74
Q

Antibodies in Sjogren’s syndrome

A

> 90% have positive Rheumatoid factor
Anti-SSA
Anti-SSB
ANA

75
Q

Non-glangular complications of Sjogren’s syndrome

A

Pancytopenia
Arthritis
lymphadenopathy (may be due to lymphatic blockage in swollen lacrimal/parotid glands)
Increased risk for lymphoma, MALT

76
Q

Antibodies positive in diffuse scleroderma

A

Anti-scl70 (DNA polymerase)
Anti-nucleolar
ANA

77
Q

Antibodies positive in local scleroderma

A

ANA

Anti-centromere

78
Q

Symptoms of diffuse scleroderma

A

Raynaud’s
Esophageal dysmotility
Scleroderma (wide range)
Telangectasias

Widespread organ sclerosis with potential for pulmonary hypertension

Fingertip pitting
Mechanic hands (seen in both Limited and diffuse)
Renal crisis

79
Q

Symptoms of limited scleroderma

A
Calcinosis
Raynaud's
Esophageal dysmotility
Scleroderma restricted to hands, feet and face
Telangectasias

Interstitial lung disease and pulm hypertension occur in much later stages than in diffuse

80
Q

Common symptoms of Polymyositis and Dermatomyositis

A
Symmetric proximal limb weakness
Difficulty swallowing with possible nasal regurg due to involvement of esophageal skeletal muscle
Lung disease
Myocarditis
Raynaud
Mechanics hands
81
Q

Features unique to polymyositis (vs dermatomyositis)

A

CD8+ (poly = more!) endomysial inflammation

82
Q

Features unique to dermatomyositis (vs polymyositis)

A
CD4+ perimysial inflammation
SKIN INVOLVEMENT
- Grotton's sign" rash on knuckles
- Periorbial rash
- Nail abnormalities

– Mechanic’s hands are seen in both

83
Q

Prognosis of dermato/polymyositis

A

Increased mortality from malignancy, infection, or pulmonary compromise

84
Q

Diagnostic criteria of dermato/polymyositis

A
CK levels should be elevated
Electromyogram abnormalities
 Anti-Jo
Anti-SRP
Anti-MI2

Not to be confused with polymyalgia rheumatica which presents similarly but has normal CK

85
Q

Treatment of dermato/polymyositis

A

Steroids for an acute flare

Long term immunosuppression

86
Q

Large vessel vasculitides

A

Giant cell arteritis

Takayasu arteritis

87
Q

Medium vessel vasculitides

A

Kawasaki

Polyarteritis nodosa

88
Q

ANCA+ Small vessel vasculitides

A

Wegener’s (granuloma polyangiitis)
Churg-Strauss (Eos polyangiitis)
Microscopic polyangiitis

89
Q

ANCA- Small vessel vasculitides (immune complex)

A

IgA vasculitis
Cryoglobulinemic vasculitis
Anti-GBM disease

90
Q

Required before beginning immunosuppression in vasculitis

A

Get biopsy. Immunosuppression can worsen many mimic diseases.

Except with suspected GCA which gets empiric high dose steroids

91
Q

Treatment options for small vessel vasculitis

A

Due to high mortality high-dose steroids are begun and tapered into a chronic immunosuppression with methotrexate or azathioprine.

If disease is life threatening, can give pulsed high dose (>1g/day) steroids with rituximab or cyclophosphamide.

92
Q

Treatment for large vessel vasculitides

A

High dose steroids

93
Q

Conditions associated with positive C-ANCA (PR3)

A

Wegner’s ONLY

Highly sensitive and specific

94
Q

Conditions associated with P-ANCA (MPO)

A

Highly sensitive, but not specific

Non-Wegner's polyangiitis  (Microscopic, Eosinophilic)
IBD
Liver disease
HIV
Lupus
95
Q

Condition also associated with GCA

A

Polymyalgia rheumatica

96
Q

Diagnostic features of takayasu’s arteritis

A

Female, asian
Narrowing of the aorta and proximal great vessels
upper extremity claudication
subclavian bruits

97
Q

Diagnostic features of polyarteritis nodosum

A

Immune complex formation secondary to hepatitis B infection–> renal micro- anyrusms
Typically affects young adults
Renal involvement can cause hypertension
Vascular purura