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DIFFUSE LUNG DISEASE: Alberto S. Santos-Ocampo, Brian F. Mandell, and Barri J. Fessler Alveolar Hemorrhage in Systemic Lupus Erythematosus : Presentation and Management Chest 2000; 118: 1083-1090 [Abstract] [Full text] [PDF]

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Alveolar hemorrhage associated with antiphospholipid syndrome

Franz J. Wiedermann   (6 November 2000)

Response to Query by Dr. Franz Wiedermann

Alberto S Santos-Ocampo   (21 November 2000)

Alveolar hemorrhage associated with antiphospholipid syndrome 6 November 2000
Franz J. Wiedermann, Consultant anesthesist Dpt. of Anaesthesia and Intensive Care Medicine, University of Innsbruck, Austria Send letter to journal: Re: Alveolar hemorrhage associated with antiphospholipid syndrome Franz.Wiedermann{at}uibk.ac.at Franz J. Wiedermann	Sir—Santos-Ocampo et al reviews seven patients with systemic lupus erythematosus (SLE) admitted with episodes of alveolar hemorrhage (AH). Recently, we published a case of multiple organ dysfunction with acute respiratory failure due to AH associated with antiphospholipid (aPL) antibodies in a 42-year-old woman with a medical history of antinuclear antibodies-negative SLE and antiphospholipid syndrome (APS)(1). The term APS was introduced to describe patients presenting with combination of recurrent arterial and venous thrombosis, recurrent fetal loss, often accompanied by thrombocytopenia, and elevations of serum aPL antibodies, e.g. lupus anticoagulant (LA) and anticardiolipin (aCL) antibodies. APS may present as a primary disorder in patients without other autoimmune diseases, or as a secondary disorder in SLE and other autoimmune diseases (2). An analysis of 29 studies yielded an average frequency of 35% for LA and 44% for aCL antibodies in SLE patients. The reported frequency of APS in SLE ranges from 20 to 35% (3). The pulmonary manifestation of APS include multiple pulmonary emboli, major pulmonary arterial thrombosis or microthrombosis with or without capillaritis, pulmonary hypertension and AH (4). The presence of aPL antibodies is not usually associated with hemorrhagic manifestations and such an event occuring in a patient with the APS is usually due to deficiency of another coagulation factor, severe thrombocytopenia or the presence of severe uremia or hepatic disease. The pulmonary vasculature, however, because of unkown factors, may be susceptible to hemorrhage (4). In the report of Santos-Ocampo et al one patient had a medical history of cerebral vascular accident from APS. We are interested to know if all patients were tested for aPL antibodies, e.g.LA and aCL antibodies or anti -ß2-glycoprotenI antibodies, and AH could also be the result of APS in some of the patients. References: 1 Wiedermann FJ, Mayr A, Schobersberger W, et al. Acute respiratory failure associated with catastrophic antiphospholipid syndrome. J Intern Med 2000;247:723-730 2 Kandiah DA, Sali A, Sheng Y, et al. Current insights into the ‘antiphospholipid’ syndrome: clinical, immunological, and molecular aspects. Adv Immunol 1998;70:507-563 3 Amigo MC, Khamashta MA, Hughes GRV. Antiphospholipid syndrome in SLE. Baillieres Clin Rheumatol 1998;12:477-493 4 Asherson RA, Cervera R. Review: antiphospholipid antibodies and the lung. J Rheumatol 1995;22:62-66 Authors: Franz J. Wiedermann MD* Wolfgang Lederer MD Wolfgang Schobersberger MD Department of Anaesthesia and Intensive Care Medicine Division of General and Surgical Intensive Care Medicine The Leopold-Franzens-University of Innsbruck Anichstrasse 35 A-6020 Innsbruck Austria * Corresponding author: Franz.Wiedermann@uibk.ac.at
Response to Query by Dr. Franz Wiedermann 21 November 2000
Alberto S Santos-Ocampo, consultant rheumatologist Makati Medical Center, Philippines Send letter to journal: Re: Response to Query by Dr. Franz Wiedermann bersanto{at}yahoo.com Alberto S Santos-Ocampo	Sir - Dr. Franz Wiedermann correctly points out that antiphospholid antibody syndrome (APS), whether primary or secondary to an underlying connective tissue disease, is known to cause alveolar hemorrhage (AH). We would like to thank Dr. Wiedermann for his interest in our article (see Electronic Letters to Santos-Ocampo AS et al. Alveolar hemorrhage in systemic lupus erythematosus: Presentation and management. Chest 2000; 118: 1083-90). In the series of 7 patients we reported, only 1 patient was known to have APS. This was discovered because she presented with a CVA at a young age. She was sub-therapeutic with her warfarin anticoagulation during both episodes of AH. However, this patient was one of the 2 in whom a transbronchial biopsy was performed at the time of AH. The histopathology of her biopsy was consistent with pulmonary capillaritis, rather than thrombosis or thromboembolism from APS. In the remaining 6 patients, a diagnosis or search for APS was not done because APS was not suggested by their clinical histories. Since many patients with SLE will have at least one positive test for APS, even without expressing the full blown syndrome, we generally avoided routine work-ups for APS unless it was warranted by the clinical situation.