Ch 3 - Rheumatology: Rheumatoid Diseases Flashcards by Carmel@Brainscape AdminPro

What is SLE?

Multisystemic, autoimmune disease that affects every organ in the body

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What is required for classification of SLE?

Positive for any 4 of 11 ACR classification criteria

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What is the classification criteria for SLE?

DOPAMINE RASH: Discoid rash
Oral ulcers
Photosensitivity
Arthritis (nonerosive)
Malar (butterfly) rash
Immunologic disorder
Neurologic disorder
Renal disorder
Abnormal ANA titer
Serositis
Hematologic disorder

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Describe malar rash.

Rash of the malar eminences that spares nasolabial folds

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Describe discoid rash.

Raised erythematous patches with keratotic scaling

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What is affected in serositis of SLE?

Pleuritis or pericarditis

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What is the MC cardiac event of SLE?

Pericarditis

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What is affected in renal disorders of SLE?

Proteinuria or cellular casts

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What neurologic disorders are seen in SLE?

Seizure or psychosis

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What hematologic disorders are seen in SLE?

Hemolytic anemia
Leukopenia
Thrombocytopenia
Lymphopenia

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Describe the arthritis of SLE.

– Small joints of the hands, wrist, and knees – Symmetric
– Migratory, chronic, nonerosive 
– Soft-tissue swelling 
– Subcutaneous nodules 
– Jaccoud’s arthritis

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What is Jaccoud’s arthritis?

Nonerosive deforming arthritis

* Ulnar deviations of the fingers and subluxations that are reversible early

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What is seen on lab work in SLE?

Dec C3 and C4
Ds-DNA
Anti-SM

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What is scleroderma?

Progressive chronic multisystem disease causing fibrosis-like changes in the skin and epithelial tissues of affected organs

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How is scleroderma classified?

By degree of skin thickening

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What are subtypes scleroderma?

Diffuse cutaneous
Limited cutaneous
Localized

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Describe features of Diffuse cutaneous scleroderma.

Affects Heart, lung, GI, kidney
Rapid onset after Raynaud’s phenomenon
Variable course—poor prognosis

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What is seen on lab work in Diffuse cutaneous scleroderma?

ANA(+)

Anti-centromere antibody (–)

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Describe features of Limited cutaneous scleroderma.

Progression after Raynaud’s phenomenon

Good prognosis

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What is seen on lab work in Limited cutaneous scleroderma?

Anti-centromere antibody (+)

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Describe features of Localized scleroderma.

Morphea

Linear scleroderma

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What is Raynaud’s phenomenon?

Vasospasm of the muscular digital arteries that can lead to ischemia and ulceration of the fingertips

What can trigger Raynaud’s phenomenon?

Cold

Emotional stresses

What is treatment of Raynaud’s phenomenon?

– Avoid cold, smoking
– Rewarming
– Ca channel blockers (nifedipine)
– EMG and biofeedback

What is Polymyositis/ Dermatomyositis?

Inflammatory myopathies involving striated muscle and clinically presents with profound symmetrical weakness of the proximal muscles

When can dysphagia occur in Polymyositis/ Dermatomyositis?

Pharyngeal involvement

Describe the clinical features of Type I primary idiopathic polymyositis.

``` – Insidious onset – Weakness starts at the pelvic girdle> shoulder girdle >neck – Dysphagia/dysphonia – Moderate-severe arthritis – Atrophic skin over knuckles – Remission and exacerbation common ```

Describe the clinical features of Type II primary idiopathic dermatomyositis.

– Acute onset – Proximal muscle weakness, tenderness – Heliotrope rash with periorbital edema – Malaise, fever, and weight loss

Describe the clinical features of Type III dermatomyositis or polymyositis.

– 5% to 8% associated with malignancy – Male, > 40 years old – Poor prognosis

Describe the clinical features of Type IV Childhood dermatomyositis or polymyositis.

– Rapid progressive weakness – Respiratory weakness – Severe joint contractures—more disabling in a child

Describe the clinical features of Type V dermatomyositis or polymyositis.

Associated with collagen vascular disease

What is seen on muscle biopsy in dermatomyositis or polymyositis?

– Perifascicular atrophy – Evidence of necrosis of type I and II fibers – Variation in fiber size – Large nuclei

What is elevated on lab work in dermatomyositis or polymyositis?

Creatinine phosphokinase Aldolase levels Transaminases LDH

What is seen on EMG in dermatomyositis or polymyositis?

– Small amplitude, short duration polyphasic motor units – Early recruitment pattern – + sharp waves, fibs – CRDs

What are dermatologic features seen in dermatomyositis?

– Lilac heliotrope rash with periorbital edema | – Gottron’s papules—scaly dermatitis over the dorsum of the hand—MCP, PIP

What are poor prognositc factors of dermatomyositis or polymyositis?

* Old age * Malignancy * Cardiac involvement * Delayed initiation of corticosteroid therapy * Respiratory muscle weakness—aspiration pneumonia * Joint contracture

What is 1st line treatment of dermatomyositis or polymyositis?

Corticosteroids: generally 1 mg/kg/day prednisone for 4 to 6 weeks, then taper

What is 2nd line treatment of dermatomyositis or polymyositis?

azathioprine or MTX

What is treatment of Refractory dermatomyositis or polymyositis?

IV immunoglobulin

Describe rehab for dermatomyositis or polymyositis?

ROM, isometric exercises—defer strengthening exercises until inflammation controlled

Describe clinical features of Juvenile dermatomyositis.

* Female>male * Heliotrope rash** * Clumsiness unrecognized * Transient arthritis * 80% to 90% respond well to corticosteroids

What is Juvenile dermatomyositis associated with?

* Generalized vasculitis | * No association with malignancy in children

What are Mixed connective tissue disorders (MCTDs)?

D/O w/ characteristics of several other diseases – SLE – Scleroderma – Polymyositis

What are overlapping symptoms of Mixed connective tissue disorders (MCTDs)?

``` – Raynaud’s phenomenon – Synovitis of the hand – Arthritis – Myopathy – Esophageal dysmotility – Acrosclerosis – Pulmonary HTN – ABN Abs ```

Which disorders are ANA (+)?

``` MCTD RA SLE Scleroderma (PSS) Polymositis Sjögren’s syndrome ```

Which disorders are RF (+)?

MCTD RA Sjögren’s syndrome

Which disorders are HLA-B27 (+)?

* AS * Reactive arthritis * Psoriatic arthritis * Enteropathic arthropathy * Pauciarticular JRA

What is Sjögren’s syndrome?

AI mediated disorder of the exocrine glands

What is the clinical presentation of Sjögren’s syndrome?

* Dry eyes * Dry mouth * Skin lesions * Parotid involvement

What is primary Sjögren’s syndrome?

Occurs in people with no other rheumatologic disorders

What is secondary Sjögren’s syndrome?

Occurs in patients with other rheumatologic disorders, most commonly RA and SLE

What are the classification features of primary Sjögren’s syndrome?

Dry eyes and mouth with ANA(+), RF (+)

What are the classification features of secondary Sjögren’s syndrome?

Sjögren’s syndrome plus evidence of SLE, RA, PSS, or polymyositis

What are extraglandular manifestions of Sjögren’s syndrome?

* Arthralgias | * Raynaud’s phenomenon