Sjogrens Syndrome

ID/CC An 18-year-old white female presents with a malar rash that is exacerbated by sun exposure {= PHOTOSENSITIVITY) as well as with arthralgias and joint stiffness involving her ankles, wrists, and knee joints; she also complains of decreased visual acuity, anorexia, weight loss, malaise, and weakness.

HPI She has a history of hematuria and no history of drug intake prior to the onset of symptoms.

PE VS: hypertension (BP 160/100). PE: pallor; malar rash; painful restriction of movement of wrist, knee, and ankle joints; no obvious deformity; whitish exudates in cytoid bodies on funduscopic exam.

Labs CBC: Coombs-positive anemia; neutropenia;

thrombocytopenia. Decreased C3, C4; positive antinuclear antibodies (ANAs), anti-native DNA, and anti-Sm antibodies; positive LE cells; false-positive VDRL due to anti-phospholipid antibodies. UA: proteinuria; RBCs and RBC casts.

XR-Plain: no erosive changes. Echo: no vegetations seen on valves (vs. endocarditis).

Serositis; pericarditis; pleuritis; splenomegaly; hyperkeratotic, erythematous plaques.

Thickening of basement membrane on renal biopsy; mesangial proliferation; thickened capillary walls, creating "wire-loop" appearance; diffuse proliferative glomerulonephritis; immune complex deposition in skin with lymphocytic infiltration; vasculitis with fibrinoid necrosis of small arteries; almost any organ may be involved.

Treatment High-dose corticosteroids for prolonged periods; alternative drugs: chloroquine; cyclophosphamide as treatment for lupus nephritis.

Discussion A type III hypersensitivity reaction. Immune complex vasculitis is the basic pathologic lesion; can be drug-induced (e.g., hydralazine, procainamide, isoniazid). p.237


Imaging Gross Pathology Micro Pathology

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Alcohol No More

Alcohol No More

Do you love a drink from time to time? A lot of us do, often when socializing with acquaintances and loved ones. Drinking may be beneficial or harmful, depending upon your age and health status, and, naturally, how much you drink.

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