MSK/Rheumatology - Rheumatology- Exam 3 Flashcards by Kelsey Peiser

What is SLE?

Chronic multi-organ autoimmune disorder, targets young adult females

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What are the clinical manifestations of SLE mediated by?

Antibody formation and creation of immune complexes that deposit in damage tissue

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Although the SLE etiology remains unknown, what are the main contributors?

Genetic, hormonal, immunologic, environmental changes that promote production of antinuclear antibodies (ANA)

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What are the general clinical presentations of SLE?

Fatigue*, fever, LAD, weight loss

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What are the skin clinical presentations of SLE?

Malar "butterfly" rash
Discoid (keratotic, photosensitivity)
Mucocutaneous - painless ulcers
Alopecia
Raynaud phenomenon

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What is the Raynaud phenomenon that occurs in 30% of patients?

Vasospastic disease of extremities with cold temps/ stress; bicentennial disease: red, white, and blue

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What is the treatment for the Raynaud phenomenon?

Calcium channel blocker

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What is discoid?

Erythematous patches with keratitis scaling in sun exposed areas

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What heart related risk is increased in SLE patients?

MI - accelerated atherosclerosis

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How can SLE affect the kidney?

Nephritis with proteinuria

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Besides systemic involvement, what other clinical manifestations are affected by SLE?

Ophthalmologic involvement, hematologic abnormalities, antiphospholipid syndrome

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What drugs are most commonly associated with drug-induced lupus?

Drugs that trigger specific immune response- procainamide, isoniazid, hydralazine

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What do patients with drug-induced lupus present with in terms of blood work?

Positive antihistone antibody

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What is the treatment for drug-induced lupus?

Stop the offending drug

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What diagnostic lab testing is performed for SLE?

Antinuclear antibody (ANA) = cardinal feature
-reported in 2 parts: titer of antibodies with serial dilution and staining pattern of antibodies
Anti-DNA
Anti-Sm
Antiphospholipid Ab

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What is a homogeneous staining pattern?

Entire nucleus stained

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What is a speckled staining pattern?

Fine or course speckles through nucleus

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What is a centromere staining pattern?

30-60 uniform speckles, localize to chromosomes in dividing cells

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What is a nucleolar staining pattern?

Homogeneous or speckled staining of nucleolus

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What are some nonpharmacologic treatments for SLE?

Sun protection
Diet/ nutrition
Exercise
Smoking cessation
Immunizations
Treatment of comorbid conditions
Pregnancy/ contraception

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What is the first line treatment for SLE?

Antimalarials = long-term daily Hydroxychloroquine (Plaquenil) with regular ophthalmologic f/u

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What meds may be added to antimalarials for the treatment for SLE?

NSAIDS, corticosteroids (systemic), immunosuppressive agents

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How many of the 11 criteria for SLE (based on the 1997 ACR criteria) do patients usually present with?

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What are the leading causes of death in patients with SLE?

Active disease (renal and CNS)
Infections
CV disease

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What is polymyositis?

Idiopathic progressive, inflammatory condition causing symmetric proximal muscle weakness, multisystem disorder

What are common complaints of a patient with polymyositis?

Weakness with deltoid and hip flexors "Difficulty getting up from a chair" "Trouble carrying groceries"

What is the clinical presentation of polymyositis?

``` Progressive symmetric muscle weakness of proximal extremities* Lungs (interstitial lung disease)* Esophageal disease Cardiac disease Raynaud phenomenon ```

What is dermatomyositis?

Polymyositis + cutaneous manifestations

What cutaneous eruptions are seen with dermatomyositis?

Heliotrope rash, Gottron's papules, Shawl sign

The link between inflammatory myopathy and what is greater in patients with dermatomyositis compared to patients with polymyositis?

Occult malignancy

What is the clinical presentation of a heliotrope rash?

Erythematous to violaceous eruption on the upper lids

What is the clinical presentation of Gottron's papules?

Erythematous/ violaceous papules on the dorsal aspect of PIP, DIP, and MCP

What is Shawl sign?

Widespread, flat, reddened area that appears on the upper back, shoulders, and back of the neck

What muscle enzymes are elevated in patients with PM and DM?

CK and aldolase

What is the 1st line of treatment of PM and DM, and what else may be added?

1st line: glucocorticoids | May add: steroid-sparing immunosuppressive agents (azathioprine, methotrexate)

What is Sjogren's syndrome (SS)?

Systemic, chronic autoimmune inflammatory disorder with exocrine gland and extraglandular features, idiopathic

What other diseases are sometimes associated with Sjogren's syndrome?

SLE, RA, systemic sclerosis

What glands are affected by Sjogren's syndrome and what is this complex called?

Lacrimal and salivary glands = "sicca complex" = combo of xerophthalmia (dry eyes) and xerostomia (dry mouth)

What is the clinical presentation of Sjogren's syndrome?

``` Constitutional (fatigue) Keratoconjunctivitis sicca- dry eyes Xerostomia- dry mouth Dryness of other surfaces Disease spectrum- extraglandular effects ```

Although there is no single diagnostic test for SS, what test may be used?

Schirmer test- test of tear production

What labs/ tests are performed for SS besides the Schirmer test?

Labs- ANA subtypes = Anti-Ro and Anti-La | Salivary gland biopsy

What is the treatment for dry eyes in SS?

``` Artificial tears Ophthalmic lubricating ointments Cyclosporine drops (Restasis) ```

What is the treatment for xerostomia in SS?

Artificial saliva Frequent sips of water Sugarless candy

What is the treatment for the extraglandular manifestations in SS?

NSAIDS and acetaminophen | Steroids and immunosuppressive medications for severe cases

What is polyarteritis nodosa (PAN)?

Systemic necrotizing vasculitis

What are the manifestations of PAN?

Transmural inflammation of muscular arteries = narrowing of lumen Results in thrombosis, ischemia, or infarct

What are not affected by PAN?

Veins

Aside from general systemic effects such as fever, weight loss, malaise, and weakness, how does PAN present clinically?

Arthralgia/ myalgia | Cutaneous effects = tender nodules, ulcers, purpura

What will a biopsy of skin lesions reveal with PAN?

Leukocytoclastic vasculitis

What is the most common systemic involvement of PAN?

Renal manifestations - infarctions and hypertension | Can also see sensory and motor polyneuropathy

Although there is no diagnostic lab test for PAN, what will be elevated in blood tests?

ESR and CRP

What will be negative in a blood test for PAN?

Antineutrophil cytoplasmic antibodies (ANCA)

What is the treatment for PAN if it is mild or cutaneous only?

Glucocorticoids

What is the treatment for PAN if it is moderate to severe?

Glucocorticoids + immunosuppressive medications

What is the treatment for PAN if it is associated with Hep B or C?

Primarily antivirals +/- glucocorticoids depending on severity

What is the treatment for PAN if it is associated with HTN?

ACE-I or ARB

Does PAN represent a spectrum of disease or a single entity?

Spectrum of disease

What is systemic sclerosis?

An autoimmune disorder that causes diffuse fibrosis (tightening and thickening) of the skin and internal organs

What vascular changes are associated with systemic sclerosis?

Narrowing of small blood vessels

Although the etiology of systemic sclerosis is unknown, what is it thought to be caused by?

Immunologic mechanisms leading to vascular endothelial damage and activation of fibroblasts

What score on the 2013 classification criteria for systemic sclerosis classifies a patient as having the definite disease?

What does CREST stand for?

``` Calcinosis- calcium deposits in skin Raynaud's phenomenon Esophageal dysfunction Sclerodactyly Telangiectasias ```

What are the characteristics of limited cutaneous SS?

CREST syndrome Skin changes limited to hands, forearm, face, neck Better prognosis

What are the characteristics of diffuse cutaneous SS?

Limited + trunk and proximal extremities (chest, abdomen, arms, shoulders) Increased risk of internal organ involvement Rapid development

What is sclerodactyly

Localized thickening/ tightening of skin (fingers, hands and toes)

What does shiny skin suggest?

Impending skin thickening

What is a common initial presentation of sclerodactyly

Diffusely puffy hands

What labs are performed for systemic sclerosis?

ANA (+) ACA (limited) Anti-Scl-70 (diffuse) Anti-RNA polymerase III (rapidly progressive diffuse)

What is the treatment for SS?

Patient education Symptomatic/ supportive care Renal and HTN: ACE-inhibitors

What treatment is given for Raynaud and esophageal disease for SS?

Raynaud- nifedipine (calcium channel blocker) | Esophageal disease- proton pump inhibitors and small, more frequent meals

What is included in patient education in treatment for SS?

Warm clothing, smoking cessation

If you see the buzz words: multi-organ, autoimmune dz, ANA, anti-dsDNA, Anti-Sm, malar rash, discoid rash, and hydroxychloroquine, what should you think of?

SLE

If you see the buzz words: peocainamide, isoniazid, hydralazine, anti-histone Ab, what should you think of?

Drug induced lupus

If you see the buzz words: progressive muscle weakness proximal UE, LE, +/- ILD, what should you think of?

Polymyositis

If you see the buzz words: transmural artery inflammation, ANCA (-), what should you think of?

Polyarteritis nodosa

If you see the buzz words: diffuse fibrosis, limited vs. diffuse, ACA, anti=SCL (anti-topoisomerase) Ab, and CREST, what should you think of?

Systemic sclerosis

If you see the buzz words: vasospasm of extremities, worsens with cold, and CCB, what should you think of?

Raynaud phenomenon

Does a + ANA automatically mean a patient has SLE?

What is pharmacologic treatment based on for SLE?

Disease severity

What is the prognosis for SLE?

Varies based on severity

What is the onset of polymyositis?

Gradual onset over weeks to months

What are other complications that a patient with polymyositis might present with?

Dysphagia, myocarditis, aspiration pneumonia

What is the major goal of treatment of PM and DM?

Improve muscle strength and avoid complications

What are general treatment recommendations for SS?

Regular f/u with dentist, ophthalmologist, rheumatologist

When PAN is triggered, what is the most typical trigger?

HBV

What is Anti-RNA polymerase III antibody associated with?

Rapidly progressive diffuse SSc, and increased risk of renal crisis