A 38-year-old woman suddenly collapses at home after experiencing severe chest pain and dies before reaching a hospital. Postmortem examination reveals an occluding thrombus overlying a ruptured atherosclerotic plaque on the left anterior descending artery. The patient is also found to have thickened mitral valve leaflets with multiple small vegetations on both valvular surfaces and fibrinoid necrosis of arterioles. Other findings include glomerular capillary basement membrane thickening with wire-loop changes. This patient was most likely suffering from which of the following conditions?

Question

A 38-year-old woman suddenly collapses at home after experiencing severe chest pain and dies before reaching a hospital.  Postmortem examination reveals an occluding thrombus overlying a ruptured atherosclerotic plaque on the left anterior descending artery.  The patient is also found to have thickened mitral valve leaflets with multiple small vegetations on both valvular surfaces and fibrinoid necrosis of arterioles.  Other findings include glomerular capillary basement membrane thickening with wire-loop changes.  This patient was most likely suffering from which of the following conditions?

Quizplus · Accepted Answer

11ec160b_38e9_a420_9249_e70e59d686e5_MD0001_00 This patient's postmortem findings are consistent with systemic lupus erythematosus (SLE), an autoimmune disease that affects women of childbearing age and results in immune complex-mediated tissue injury in various organs. Cardiovascular manifestations of SLE include accelerated atherosclerosis with increased risk of myocardial infarction. Small arteries and arterioles can also show evidence of vasculitis with fibrinoid necrosis in any tissue. Other cardiac features include pericarditis and less commonly verrucous (Libman-Sacks) endocarditis, characterized by thickened valve leaflets (typically mitral [red arrows] and/or aortic [black arrows]) with multiple small vegetations on both surfaces. The vegetations are composed of sterile platelet thrombi intertwined with strands of fibrin, immune complexes, and mononuclear cells. These vegetations are easily dislodged and can result in systemic embolization. Renal involvement in SLE most often manifests as diffuse proliferative glomerulonephritis, which is characterized by proliferative and necrotizing lesions with crescent formation during active disease. Light microscopy also classically shows diffuse thickening of the glomerular capillary walls with "wire-loop" structures due to subendothelial immune complex deposition. (Choice A) Bacterial endocarditis is characterized by large, potentially destructive vegetations on the valve cusps that contain fibrin, inflammatory cells, and bacteria (eg, staphylococci, streptococci). Renal involvement can occur with septic embolization or development of immune complex-mediated glomerulonephritis with hypercellular infiltrate and immune deposits in the glomerular capillary wall. (Choice B) Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis) is a small-vessel necrotizing vasculitis characterized by allergic rhinitis, asthma, and peripheral eosinophilia. Although renal involvement is not typically prominent, patients can develop necrotizing crescentic glomerulonephritis. (Choice C) Granulomatosis with polyangiitis (Wegener granulomatosis) is a small-vessel necrotizing vasculitis characterized by upper respiratory (eg, sinusitis, otitis media) and lower respiratory (eg, dyspnea, hemoptysis) symptoms as well as renal disease. Renal involvement typically manifests as focal necrotizing, crescentic glomerulonephritis. (Choice E) Thromboangiitis obliterans (Buerger disease) is a small- to medium-vessel vasculitis that typically occurs with cigarette smoking and causes vascular insufficiency in the extremities (eg, gangrene, autoamputation of digits). It is characterized histologically by intraluminal thrombus and prominent inflammatory cell infiltrate with relative sparing of the vessel wall. Educational objective: Cardiovascular manifestations of lupus include accelerated atherosclerosis, small-vessel necrotizing vasculitis, pericarditis, and Libman-Sacks endocarditis (small, sterile vegetations on both sides of the valve). Renal involvement classically manifests as diffuse proliferative glomerulonephritis, which is characterized by diffuse thickening of the glomerular capillary walls with "wire-loop" structures on light microscopy. __________ References: The classification of glomerulonephritis in systemic lupus erythematosus revisited. (http://www.ncbi.nlm.nih.gov/pubmed/14747370) Diagnostic histopathology of major systemic and pulmonary vasculitis syndromes. (http://www.ncbi.nlm.nih.gov/pubmed/1971725)

A 38-Year-Old Woman Suddenly Collapses at Home After Experiencing Severe