Chronic catastrophic-like antiphospholipid syndrome: a “smoldering” variant?
ABSTRACT Catastrophic antiphospholipid syndrome (CAPS) is characterized by life-threatening diffuse thrombotic manifestations involving particularly small vessels of kidney, lungs, brain and skin. We report a 20-year-old female with systemic lupus erythematosus and secondary antiphospholipid syndrome who presented typical organ and histological involvement as seen in CAPS but with protracted course suggesting a “smoldering” form of the disease.
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ABSTRACT: Pulmonary manifestations in antiphospholipid syndrome (APS) are relatively rare compared to other clinical signs of this disease. However, pulmonary microthrombosis is among the most frequent arterial complications of APS. Timely diagnosis of pulmonary manifestations is required due both to their severity and to the high mortality rate. The spectrum of pulmonary disorders in patients with antiphospholipid antibodies is sometimes referred to as the "antiphospholipid lung syndrome", and includes thromboembolism of lung arteries, pulmonary hypertension, adult respiratory distress syndrome, intra-alveolar hemorrhages, postpartum syndrome, and others. Timely diagnosis and correct therapy, as well as good patient-doctor collaboration, can result in stabilization of the patient's condition.Autoimmunity Reviews 06/2006; 5(5):344-8. · 7.10 Impact Factor
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ABSTRACT: Pulmonary vascular inflammatory disorders may involve all components of the pulmonary vasculature, including capillaries. The principal histopathologic features of pulmonary capillaritis include capillary wall necrosis with infiltration by neutrophils, interstitial erythrocytes, and/or hemosiderin, and interalveolar septal capillary occlusion by fibrin thrombi. Immune complex deposition is variably present. Patients often present clinically with diffuse alveolar hemorrhage, which is characterized by dyspnea and hemoptysis; diffuse, bilateral, alveolar infiltrates on chest radiograph; and anemia. Pulmonary capillaritis has been reported with variable frequency and severity as a manifestation of Wegener's granulomatosis, microscopic polyarteritis, systemic lupus erythematosus, Goodpasture's syndrome, idiopathic pulmonary renal syndrome, Behçet's syndrome, Henoch-Schönlein purpura, IgA nephropathy, antiphospholipid syndrome, progressive systemic sclerosis, and diphenylhydantoin use. In addition to history, physical examination, and routine laboratory studies, certain ancillary laboratory tests, such as antineutrophil cytoplasmic antibodies, antinuclear antibodies, and antiglomerular basement membrane antibodies, may help diagnose an underlying disease. Diagnosis of pulmonary capillaritis can be made by fiberoptic bronchoscopy with transbronchial biopsy, but thoracoscopic biopsy is often employed. Since many disorders can result in pulmonary capillaritis with diffuse alveolar hemorrhage, it is crucial for clinicians and pathologists to work together when attempting to identify an underlying disease. Therapy depends on the disorder that gave rise to the pulmonary capillaritis and usually includes corticosteroids and cyclophosphamide or azathioprine. Since most diseases that result in pulmonary capillaritis are treated with immunosuppression, infection must be excluded aggressively.Chest 12/1996; 110(5):1305-16. · 7.13 Impact Factor
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ABSTRACT: To discuss the clinical manifestations and possible pathogenic mechanisms of the unusual syndrome of diffuse alveolar hemorrhage (DAH) and pulmonary capillaritis without thrombosis in the setting of the primary antiphospholipid antibody syndrome (PAPS). Four men with DAH and capillaritis in the setting of PAPS are identified. Their clinical presentations, laboratory, radiographic, and pathologic findings are reviewed as is their clinical course and response to therapy. In addition, the literature regarding DAH and pulmonary capillaritis in the setting of PAPS is reviewed. The patients presented with dyspnea, hemoptysis, fever, hypoxia, and diffuse alveolar infiltrates; none had evidence of acute thromboembolic disease. All secondary causes of DAH were ruled out. All patients had positive testing for the lupus anticoagulant and high-titer anticardiolipin antibodies, including antibodies against the beta-2-glycoprotein I antigen. Three cases had lung biopsies that revealed pulmonary capillaritis and DAH with no evidence of thrombosis. All patients improved with high-dose corticosteroids. Recurrent disease in the setting of aggressive immunosuppression responded to intravenous immunoglobulin. Antiphospholipid antibody-mediated endothelial cell activation in the absence of thrombosis may induce capillaritis as seen in these cases. The syndrome of DAH and pulmonary capillaritis is further defined. Evidence supports a causative relationship between PAPS, pulmonary capillaritis, and DAH in the absence of thromboembolic disease. Further elucidation of a possible nonthrombotic mechanism of antiphospholipid antibody-mediated pathology is needed to guide future therapies for this unusual manifestation of PAPS.Seminars in Arthritis and Rheumatism 01/2006; 35(3):154-65. · 3.63 Impact Factor