Connective tissue diseases. Flashcards
Associated findings with ANA-negative lupus
Arthritis, Raynaud’s, subacute cutaneous lupus
Ro (anti-SS-A) Ab +, ANA -
Risk of neonatal lupus in infants of affected women
Clinical findings associated with neonatal lupus
Skin lesions Cardiac abnormalities (AV block, transposition of the great vessels) Valvular and septal defects
Most prominent finding in most patients with SLE
Fatigue – often sign of impending exacerbation
Most common cardiac finding in SLE
Pericarditis
Hematoxylin bodies are found in what pathology?
Libman Sacks Endocarditis – only difference between this and nonbacterial thrombotic endocarditis. Bodies are found in areas of focal necrosis.
Which Abs are peresent in drug-induced lupus?
Anti-histone Abs. Its sensitive but not specific for drug-induced lupus.
P-ANCA is positive in what condition?
Polyarteritis nodosa
Some commonly implicated agents in drug-induced lupus
Hydralazine, Procainamide, Isoniazid, Chlorpromazine, methyldopa, and quinidine.
Best long term tx for SLE
antimalarials like hydroxychloroquine for constitutional, cutaneous, and articular manifestations. Is continued as preventative measure even after resolution of sx but annual eye exam is needed due to retinal toxicity.
How do you treat SLE glomerulonephritis?
Cytotoxic agents such as cyclophosphamide
Mechanisms of anemia in SLE
Anemia of chronic disease, renal insufficiency from glomerulonephritis, iron deficiency anemia (GI loss due to meds like NSAIDs), autoimmune hemolytic anemia. Can also be caused by medications, hypersplenism, microangiopathic hemolytic anemia, and aplastic anemia.
Mechanisms of leukopenia in SLE
Autoimmune mediated destruction. Uncommonly due to medications, hypersplenism, or bone marrow dysfunction.
Mechanisms of thrombocytopenia in SLE
Immune mediated destruction. Uncommonly due to medications or increased consumption due to thrombotic microangiopathy (TTP)
Pathophys of scleroderma renal crisis
Occurs in up to 20% of pts with diffuse cutaneous systemic sclerosis. Increased vascular permeability, activation of the coat cascade and increased renin secretion causes pts to develop sudden onset of renal failure and malignant HTN. UA can be normal or show mild proteinuria. Peripheral blood smear can show microangiopathic hemolytic anemia with fragmented red blood cells and thrombocytopenia
SE of hydroxychlorquine
retinopathy. need eye exams q 6 months
Which form of PM/DM have the worst long term prognosis?
Those with antisynthetase Ab are often associated with ILD, other extra muscular mx, and a worse long term prognosis due to the related lung disease.
Which agent is preferred in addition to glucocorticoids in patients who have ILD associated with myositis?
Azathioprine. Also preferred if pt has underlying liver dz or unwilling to abstain from alcohol.
SE of azathioprine
Systemic flu like run associated with fever and GI complaints. Other side effects includee bone marrow suppression, pancreatitis, and liver toxicity. Long term may include increased risk of malignancy.
