-women, 35-40

-No renal involvement

-arthralgias, Raynaud phenomoneon, esophageal hypomotility, myositis

-anti-nRNP, speckled nuclear appearance

-more common in men

-affects small and medium vessels

-segmental, fibrinoid, necrosis

-an immune complex vasculitis

-the antigen (which causes the formation of antibodies) could be Hepatitis B or drugs (sulfonamides and penicillin)

-if lung involved à chest pain, cough, dyspnea, eosinophilia

-pregnant women

-proximal muscles of extremities

-skin can get involved (red-purple rash) à dermatomyositis

-increased serum creatine kinase

-muscle biopsy à necrotic muscle cells, lymphocytic infiltrate

-widespread fibrosis and degenerative changes involving many organs

-fixed facial appearance, sclerodactyly (claw-like hand), Raynaud phenomenon, visceral organs involvement (esophagus-dysphagia, lung-internsitial pulmonary fibrosis, hypertension).

-anti-Scl-70, ANAs with anticentromere activity (in CREST syndrome)

-CREST syndrome: caclinosis, raynaud phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia

-women

-Triad: dry mouth, dry eyes, rheumatoid arthritis

-Sicca syndrome: only dry mouth and dry eyes

-infiltration by lymphocytes and plasma cells of the Parotids à obscure parenchyma of parotid gland à malignant lymphoma

-hypergammaglobulinemia, anit-SS-B, anti-SS-A

-women of childbearing age

-join problems

-butterfly rash

-Raynaud

-serosal: pericarditis, pleuritis

-diffuse interstitial pulmonary fibrosis: interstitial pneumonitis, diffuse fibrosing alveolitis

-verrucous endocarditis – affects mitral valves on both sides

-vasculitis – onion skin appearnce (concentric rings of collagen)

-Glomerular changes

            -immune complex deposition: subendothelial (wireloop), mesangial

            -endothelial proliferation

            -thickening basement memberane

-Diagnosis: LE cells, dsDNA, Sm antigen, decreased serum complement, immun complexes at dermal-epidermal junction, false-positive test for syphilis (due to cardiolipin)