Ch 3 - Rheumatology: Rheumatoid Diseases Flashcards
What is SLE?
Multisystemic, autoimmune disease that affects every organ in the body
What is required for classification of SLE?
Positive for any 4 of 11 ACR classification criteria
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
Describe malar rash.
Rash of the malar eminences that spares nasolabial folds
Describe discoid rash.
Raised erythematous patches with keratotic scaling
What is affected in serositis of SLE?
Pleuritis or pericarditis
What is the MC cardiac event of SLE?
Pericarditis
What is affected in renal disorders of SLE?
Proteinuria or cellular casts
What neurologic disorders are seen in SLE?
Seizure or psychosis
What hematologic disorders are seen in SLE?
Hemolytic anemia
Leukopenia
Thrombocytopenia
Lymphopenia
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
What is Jaccoud’s arthritis?
- Nonerosive deforming arthritis
* Ulnar deviations of the fingers and subluxations that are reversible early
What is seen on lab work in SLE?
- Dec C3 and C4
- Ds-DNA
- Anti-SM
What is scleroderma?
Progressive chronic multisystem disease causing fibrosis-like changes in the skin and epithelial tissues of affected organs
How is scleroderma classified?
By degree of skin thickening
What are subtypes scleroderma?
Diffuse cutaneous
Limited cutaneous
Localized
Describe features of Diffuse cutaneous scleroderma.
Affects Heart, lung, GI, kidney
Rapid onset after Raynaud’s phenomenon
Variable course—poor prognosis
What is seen on lab work in Diffuse cutaneous scleroderma?
ANA(+)
Anti-centromere antibody (–)
Describe features of Limited cutaneous scleroderma.
Progression after Raynaud’s phenomenon
Good prognosis
What is seen on lab work in Limited cutaneous scleroderma?
Anti-centromere antibody (+)
Describe features of Localized scleroderma.
Morphea
Linear scleroderma
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
