1/3403. Glomerular thrombosis: an unusual cause of renal failure in systemic lupus erythematosus. The authors report an unusual case of acute renal failure occurring in a patient with systemic lupus erythematosus and antiphospholipid antibodies. kidney biopsy revealed glomerular thrombosis, in the absence of glomerulonephritis. The authors stress the clinical and biological signs that suggest the thrombotic nature of kidney failure in lupus patients. ( info) |
2/3403. 'Sustained remission' in a case of SLE following megadose cyclophosphamide. Cytotoxic therapy, especially with cyclophosphamide in the dose 8-20 mg/kg used as intermittent pulses, has been shown to improve both patient and renal survival in systematic lupus erythematosus (SLE), but to date there is no cure for the disease. Owing to the paucity of recognisable clones, the rationale and goal of cytotoxic immunosuppressive therapy in the treatment of immune-mediated diseases as against malignancies is to suppress the aberrant inflammation and immune-mediated reactions responsible for tissue damage, without dangerously suppressing the normal host defence mechanism(s). We report the case of a patient suffering from SLE with nephritis who has remained in sustained remission over the past 8 years without any maintenance therapy following an accidental administration of a single dose of 5000 mg of intravenous cyclophosphamide (44.2 mg/kg body weight). The patient recovered fully from pancytopenia following the injection. Presently, she is asymptomatic and working gainfully. Her laboratory parameters including blood counts, urine analysis, FANA and anti-dsDNA have reverted to normal. cyclophosphamide in the dose of 30-160 mg/kg has been safely and effectively used in various neoplastic conditions with the aim of destroying every possible tumour cell. The experience of the present case suggests that such an approach may be applicable to SLE. ( info) |
3/3403. pseudotumor cerebri and leukoencephalopathy in childhood lupus. We describe an adolescent with systemic lupus erythematosus (SLE) and pseudotumor cerebri (PTC) associated with diffuse white matter lesions (leukoencephalopathy) on neuroimaging studies. Although the association between SLE and PTC has been reported previously in 21 cases, the findings of leukoencephalopathy is known in only one other patient. ( info) |
4/3403. Cutaneous mycobacterium kansasii infection in a patient with systemic lupus erythematosus: case report and review. mycobacterium kansasii infections of the skin have been described in 31 previously published cases. The median age of these patients is 43 years, and male patients are more frequently affected than female patients. Most patients (72%) with this infection have some alteration of their immune status, but disseminated infection is relatively uncommon (22%). We present the first reported case of cutaneous M. kansasii infection in a patient with previously diagnosed systemic lupus erythematosus. The clinical presentation is similar to that expected in lupus profundus. While the duration of treatment is long (18 months), this case demonstrates that rifampin combined with at least 2 other antibiotics can provide excellent results. clarithromycin has demonstrated encouraging in vitro results against M. kansasii but has not yet been reported for treatment of cutaneous infections. ( info) |
5/3403. Hemophilus influenzae biotype III cellulitis in an adult. The case of a patient with systemic lupus erythematosus presenting with severe leg cellulitis caused by Hemophilus influenzae non-B biotype III is reported. skin infections caused by H. influenzae in general, and of the extremities in particular, seem to be rare in adults. This is the first reported case of cellulitis caused by H. influenzae biotype III. The infection was treated successfully with antibiotics. This case highlights the importance of blood cultures and prompt antimicrobial treatment in febrile adults with cellulitis, especially immunocompromised patients. ( info) |
6/3403. Lupus anticoagulant in myelofibrosis. A patient with myelofibrosis who also demonstrates a lupus anticoagulant is reported. The presence of a circulating anticoagulant adds to the list of potential hemorrhagic diatheses in myelofibrosis and also demonstrates myelofibrosis to involve a system that may be separate from the myeloid elements of the bone marrow. ( info) |
7/3403. Valvular deposition of antiphospholipid antibodies in the antiphospholipid syndrome: a clue to the origin of the disease. In this report we present an unusual case of a 45-year-old female patient with systemic lupus erythematosus (SLE) who was hospitalized for mitral valve replacement. In her childhood she presented with mitral stenosis and chorea on which grounds a preliminary diagnosis of rheumatic fever was established. After a quiescent period lasting two decades her disease erupted with mitral stenosis, thromboembolic phenomena, and nephritis. Due to severe malfunctioning of her mitral valve, the patient eventually underwent mitral valve replacement. The antibodies involved in the pathogenesis of our patient's valvular disease were studied by immunohistochemical analysis, applying rabbit polyclonal anti-human IgG and IgM anti-human C3c and anti-idiotypes to a mouse monoclonal naturally occurring polyspecific human monoclonal anti-cardiolipin antibody termed S2.9, and to the 16/6 Id which defines a common Id on anti-dna antibodies in patients with SLE. Immunoperoxidase staining using an anti-idiotype mAb to anti-cardiolipin antibodies demonstrated the deposition of these anti-bodies in the subendothelial layer of the valve. We believe that anti-phospholipid syndrome (APS) with SLE was the initial and primary disease in this patient. These findings clearly indicate that APS must be considered in the differential diagnosis of rheumatic fever, particularly in young female patients who present with mitral stenosis and chorea. ( info) |
8/3403. Systemic lupus erythematosus with optical neuromyelitis (Devic's syndrome). A case with a 35-year follow-up. Optical neuromyelitis or Devic's syndrome is a very uncommon neurological manifestation of systemic lupus erythematosus. It is also associated with antiphospholipid antibodies, limited responsiveness to glucocorticoid treatment and a poor prognosis. We report the case of a female systemic lupus erythematosus patient who developed recurrent flares of optical neuritis and transverse myelitis. These flares consistently responded to glucocorticoid therapy. Despite the absence of overt anticardiolipin antibodies in the course of the disease, long-term anticoagulant therapy has been introduced with positive results. Treatments are usually of limited efficacy in Devic's syndrome. In our patient, however, aggressive glucocorticoid treatment resulted in prolonged survival. ( info) |
9/3403. Myocardial infarction in patients with systemic lupus erythematosus with normal findings from coronary arteriography and without coronary vasculitis--case reports. The authors present the cases of two young patients, a man and a woman, who presented with myocardial infarction, in the absence of ischemic heart disease or stenosis of the coronary arteries. The woman was known to have systemic lupus erythematosus (SLE) for the past 3 years (the immunoglobulin m [IgM] anticardiolipins antibodies were positive), without a history of coronary risk factors. Suddenly she presented with acute chest pain on rest that lasted 4 hours and culminated in anterior wall myocardial infarction. She was admitted to the coronary care unit, where no thrombolysis was given. She did not have echocardiographic evidence of Libman-Sacks endocarditis, but myocardial infarction was evident at the electrocardiogram (ECG). The young man had SLE (the IgM anticardiolipins were absent, but he was positive for lupus anticoagulant antibodies), he was hyperlipidemic, was a moderate smoker and moderately obese, and had no history of ischemic heart disease. He suddenly presented with an acute myocardial infarction documented by ECG, enzymes, and gammagraphy. In both patients, coronary angiography findings were normal and myocardial biopsy did not show evidence of arteritis. The relevance of these cases is the rare association of ischemic heart disease in SLE, with normal coronary arteries and without evidence of arteritis or verrucous endocarditis. ( info) |
10/3403. Acquired C1 inhibitor deficiency associated with systemic lupus erythematosus, secondary antiphospholipid syndrome and IgM monoclonal paraproteinaemia. In this case report, a woman is described who developed systemic lupus erythematosus at the age of 36 years affecting the central nervous system and kidneys, showing the serological evidence of an acquired C1 inhibitor deficiency, but also developing an antiphospholipid syndrome and IgM lambda type paraproteinemia. During the 25 years follow-up she did not show any signs of angioedema. Although the immunological abnormalities are still constantly present, the disease has been quiescent since the past three years. The possible pathophysiology of clinical and laboratory features are discussed. ( info) |