Autoimmune Diseases Flashcards
1
Q
Systemic Lupus Erythematosus (SLE)
Diagnostic Criteria
A
4 or more of DOPAMINE RASH
- Discoid rash
- Oral ulcers
- Photosensitivity
- Arthritis (symmetric with no deformity)
- Malar rash
- Immunologic criteria (Anti-Ds DNA, Anti-Sm,
- Neurologic symptoms (lupus cerebritis, seizures, psychosis and stroke)
- Elevated ESR
- Renal disease (diffuse proliferative GN [nephritic, subendothelial deposits, wire-loop] is the most common and severe, second common is membranous GN)
- ANA +ve
- Serositis (pleural or pericardial effusion)
- Hematologic abnormalities (hemolytic anemia, anemia of chronic disease, leukopenia and thrombocytopenia)
2
Q
Systemic Lupus Erythematosus (SLE)
Diagnostic Tests
A
- ANA extremely sensitive
- Anti-Ds DNA (60%) and Anti-Sm (30%) are extremely specific
- Anti-Histone antibodies are specific for drug-induced lupus (hydralazine, INH, and procainamide)
- Anti-SSA (Ro) and Anti-SSB (La) found in 10-20%
- Anti-Ro antibodies associated with neonatal SLE
- Decreased complement levels and rise in anti-Ds DNA antibodies in an acute lupus exacerbations
- Renal biopsy to determine the type of GN
3
Q
Systemic Lupus Erythematosus (SLE)
Important facts and Associations
A
- All manifestations are type III hypersensitivity reaction except for hematologic ones are type II
- Most common in African-Americans, women in their reproductive age
- Patients die from infection, renal failure or MI (now is most common because of accelerated atherosclerosis due to vasculitis)
- Associated with mesenteric vasculitis, Raynaud phenomenon, Antiphospholipid syndromes, and Libman-Sacks endocarditis (non-infectious vegetations often seen on both sides of mitral valve)
4
Q
Systemic Lupus Erythematosus (SLE)
Treatment
A
- Mild joint pain: NSAIDs
- Isolated skin or joint involvement: hydroxychloroquine
- Acute exacerbations: high-dose boluses of corticosteroids
- Lupus nephritis: corticosteroids either alone or with cyclophosphamide or mycophenolate
- Progressive or refractory cases: corticosteroids, hydroxychloroquine, cyclophosphamide and azathioprine
- Control progression: Belimumab
5
Q
Sjögren syndrome (Sicca syndrome)
Presentation
A
- Dry mouth (need to constantly drink water and dysphagia, with rampant dental caries)
- Dry eyes (“sand in eyes” feeling, burning and itching. it is called keratoconjunctivitis sicca)
- Dry vagina (dyspareunia)
- Less common presentations are
- Vasculitis
- Lung disease
- Pancreatitis
- Renal tubular acidosis (20%)
6
Q
Sjögren syndrome (Sicca syndrome)
Associations
A
- Non-Hodgkin lymphoma ( 10% of cases and most dangerous complication)
- Rheumatoid arthritis
- SLE
- Primary biliary cirrhosis
- Polymyositis
- Hashimoto thyroiditis
7
Q
Sjögren syndrome (Sicca syndrome)
Diagnostic Tests
A
- Best initial test is Schirmer test (filter paper placed against the eye)
- Best initial test on blood is Anti-SSA (Ro) and Anti-SSB (La)
- Most accurate test is lip or parotid gland biopsy (lymphoid infiltration)
- Rose bengal stain shows abnormal corneal epithelium
8
Q
Sjögren syndrome (Sicca syndrome)
Treatment
A
- Best initial therapy is to water mouth (frequent water sips, sugar-free gum, and fluoride)
- Artificial tears
- Pilocarpine and cevimeline (increase ACh which is the main stimulant of saliva production)
9
Q
Scleroderma
Pathogenesis
A
- A progressive autoimmune disease with unknown cause
- Can be presented as diffuse scleroderma or localized scleroderma (CREST syndrome)
- Fibroblasts stimulation by cytokines (IL-1, PDGF, FGF, and TGF-beta) which lead to deposition of type I and III collagen in skin and internal organs (lungs, heart, GI, and kidneys)
- Death is usually from pulmonary hypertension or its complications
10
Q
Scleroderma (Diffuse Scleroderma)
Presentation
A
20% of cases
- Widespread skin involvement (hands, face, neck and extremities and abnormalities of pigmentation)
- Esophagus (dysphagia), GI (malabsorption and large-mouthed diverticuli of small and large bowel)
- Lungs (pulmonary fibrosis [restrictive lung disease] with dyspnea on exertion and pulmonary hypertension)
- Heart (myocardial fibrosis, pericarditis, heart block and lung disease gives RV hypertrophy)
- Kidneys (fibrosis which leads to sudden onset hypertensive crisis and renal insufficiency)
11
Q
Scleroderma (Localized Scleroderma)
Presentation
A
CREST syndrome (80% of cases)
- Calcinosis
- Raynaud syndrome: increreased vascular reactivity of fingers beginning with pain and pallor (white) or cyanosis followed by hyperemia (red). Precipitated by cold and emotional stress. Some cases lead to ulceration and gangrene
- Esophageal dysmotility: presented as dysphagia and GERD
- Sclerodactyly
- Telangiectasias
12
Q
Scleroderma
Diagnostic Tests
A
- ANA is sensitive but not specific
- Scl-70 (anti-DNA topoisomerase I antibodies): the most specific test but present only in 30% of diffuse and 20% of localized
- Anti-centromere antibodies present in 50% of CREST (extremely specific)
13
Q
Scleroderma
Treatment
A
- Raynaud syndrome: calcium channel blockers
- Esophageal dysmotility: PPIs for GERD
- Acute flares: corticosteroids
- Renal crisis: ACE inhibitors (use even if creatinine is elevated)
- Pulmonary fibrosis: cyclophosphamide improves dyspnea and PFTs
- Pulmonary hypertension:
- Bosentan ambrisentan (endothelin antagonist)
- Sildenafil
- Prostcyclin analogs (iloprost, treprostinil and epoprostenol)
- Methotrexate slows underlying disease process of CREST
- Penicillamine can be used for skin changes
- Bleomycin and Amiodarone should never be given in diffuse scleroderma (they cause pulmonary fibrosis)
14
Q
Polymyositis
Pathogenesis
A
- Progressive, systemic connective tissue disease characterized by immune-mediated striated muscle inflammation (cytotoxic T lymphocytes)
- Unknown cause
- Associated with nasopharyngeal cancer, lung cancer, non-Hodgkin lymphoma and bladder cancer
15
Q
Dermatomyositis
Pathogenesis
A
- Progressive, systemic connective tissue disease characterized by immune-mediated inflammation of skin and muscles (humorally mediated angiopathy resulting in myositis and dermatitis)
- Unknown cause
- Inherited in some cases with HLA-DR3, HLA-DR52 and HLA-DR6
- In 25% of cases associated with ovarian cancer, breast cancer, lung cancer, GI cancer and lymphoma
