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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Systemic lupus erythematosus (SLE) is not a rare disease. There are several common clinical signs which should alert the physician to a possible diagnosis of SLE and which should condition him to look for specific clinical and laboratory findings. In addition to simple screening tests, useful procedures include a search for antinuclear antibodies, lupus erythematosus (LE) cells, anti-DNA antibodies and low serum complement. Management is determined by the type of course encountered but most patients will do well under the care of their family physician.
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PMID:Diagnosis and management of systemic lupus erythematosus. 7 45

Pulmonary hypertension due to recurrent thromboembolism is a rare disease but life-threatening. We evaluated 18 patients (11 female, 7 male) with this pathology between 1973 and 1991. We compared clinical features and evolution of our patients with the ones of the literature. The mean interval between beginning of symptoms and diagnosis was 5 years (range 1-10 years) and the most frequent symptom was increasing dyspnoea. In 2 of our patients there were well definite predisposing causes for thromboembolism (intracardiac catheters), 6 of the others had a previous episode of acute pulmonary embolism. Mean pulmonary arterial pressure was 50 mmHg and low output was present in 8 of these. Lung perfusion scintigraphy was diagnostic in 98% of cases showing segmental defects and pulmonary angiography confirms diagnosis revealing abrupt cut-off of cases showing segmental defects and pulmonary angiography confirms diagnosis revealing abrupt cut-off a major pulmonary artery. Angiographic evaluation of thrombus extent and location was difficult. In a small number of patients was found lupus anticoagulant, deficiency of protein C, of protein S and of antithrombin III. Mortality in medical treatment was 39% at a mean follow-up of 4-5 years. Progression of pulmonary hypertension was due to recurrent pulmonary embolism only in 30-40% of cases. The role of caval filter is not well established. Thromboendarterectomy shows immediate good results at short time but the long-term results are not known.
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PMID:[Thromboembolic pulmonary hypertension]. 184 71

Angioedema is characterized by localized swelling of sudden onset affecting the skin and/or mucous membranes. It can be classified into hereditary and acquired forms. Hereditary angioedema is a rare disease inherited as an autosomal dominant trait and caused by a deficiency of C1-esterase inhibitor. Acute attacks are life threatening and cannot be managed by antihistamines, corticosteroids, or adrenergic drugs. Prophylactic therapy is possible with danazol or stanozolol. Acquired angioedema includes nonhereditary C1-esterase inhibitor deficiency; idiopathic, allergic, and drug-induced forms; angioedema associated with lupus erythematosus and hypereosinophilia; and angioedema caused by physical stimuli. Treatment of these forms of angioedema depends on identifying and avoiding the cause, induction of tolerance, or symptomatic treatment with systemic antihistamines.
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PMID:Angioedema: manifestations and management. 186 90

A patient is described with stable primary biliary cirrhosis and recurrent gastrointestinal bleeding due to angiodysplasias in the gastrointestinal tract. This is a very rare disease association. In addition this patient showed two other disease associations, not known in the literature. She also suffered from eosinophilic colitis and had the lupus anticoagulant. These associations have not yet been described in the literature.
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PMID:Primary biliary cirrhosis associated with recurrent angiodysplastic lesions in the gastrointestinal tract, the lupus anticoagulant and eosinophilic colitis. 196 45

Relapsing polychondritis is a rare disease of uncertain etiology, characterized by inflammation of cartilage. Cartilage of the respiratory tract is engaged in more than half of all cases. Hydralazine-induced systemic lupus erythematosis (SLE) is a well known disease. Females who are slow acetylators and are HLA-DR4 positive when treated with hydralazine are at serious risk of contracting SLE syndrome. We describe here a woman, treated for 10 years with hydralazine, who was a slow acetylator and was HLA-DR4 positive and who presented with a relapsing polychondritis and ultimately required a permanent tracheostoma. To our knowledge, this is the first published case of relapsing polychondritis induced by hydralazine.
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PMID:Hydralazine-induced relapsing polychondritis-like syndrome. Report of a case with severe chronic laryngeal complications. 660 52

Lichen planus belongs to the group of frequent skin diseases. Therefore, the concurrence with other diseases might be expected by chance. However, the bullous form of lichen planus is a rare disease and for this variant a coincidence with malignant diseases, tumors and systemic diseases, has been reported. Additionally, in the last decade the association of lichen planus respectively lichen planus pemphigoides and diseases accompanied with or caused by immunologic dysfunction has been pointed out. This concerns lupus erythematosus, myasthenia gravis, and ulcerative colitis, diseases, which are accompanied by circulating organ-specific antibodies. A further patient is reported who developed a lichen planus pemphigoides consecutively to an ulcerative colitis.
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PMID:[Concurrence of lichen planus pemphigoides and ulcerative colitis (author's transl)]. 722 95

The present study describes the epidemiological profile and clinical features of Takayasu's arteritis (TA) in Kuwait, as well as its association with other autoimmune diseases and primary hypercoagulable states. Thirteen patients were included from its start on 1 January 1989 till 30 June 1994. Diagnosis of TA was established by angiographic studies. Twelve patients were Arabs and 7 were Kuwaiti nationals. Five patients were males and renal disease secondary to isolated involvement of the abdominal aorta (TA, type II) was the main presentation in 4 patients. Coagulation tests were performed in 7 patients and included antiphospholipid antibody (aPL) assay as well as protein S, protein C and antithrombin III activity. Only one manifested recurrent thrombosis and laboratory tests confirmed the presence of a hypercoagulable state secondary to aPL and protein S deficiency. Serological tests of systemic lupus erythematosis (SLE) were positive in this patient. These data indicate that TA is not a rare disease in the Arabic population. In our study, female predominance was not a common feature of TA and renal disease secondary to TA type II disease was commonly encountered. The association of TA with SLE and primary hypercoagulable states was not a consistent finding in our patients with TA, and hence, the proposed role for thrombotic vasculopathy in the pathogenesis and progression of this disease was unfounded.
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PMID:Takayasu's arteritis in Kuwait. 756 55

Sneddon's syndrome is a rare disease characterised by cerebrovascular ischaemic attacks and generalised livedo. Since previous observations, other symptoms were described: involvement of heart, of kidney, arterial hypertension, complicated obstetric or gynaecologic history in women. Prognosis is highly variable, depending on extent and speed of progression of cerebrovascular changes, which can lead to severe permanent mental deterioration. In livedo, histopathology shows pathological changes of small to medium-sized dermal arteries in a distinct time sequence: an early phase localized in endothelium followed by a late fibrotic phase. No specific laboratory findings are found. Recently some cases were reported in association with antiphospholipids antibodies. The etiopathogeny of Sneddon's syndrome is still unknown and could result from different processes: progression to an autoimmune disease such as lupus erythematosus, primitive endarteritis obliterans, or a new clinical expression of the antiphospholipid antibodies syndrome. At present, none of the therapeutic modalities provides significant improvement.
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PMID:[Sneddon syndrome]. 770 55

Kikuchi's disease is rare necrotic histiocytosis of the lymph nodes. Since the first description in Japan in 1972 by Kikuchi, several cases have been reported. The disease occurs most frequently in young women. Manifestations include enlargement of the cervical lymph nodes, sometimes with fever, and often associated with other non-specific clinical signs. Blood chemistry, including immunologic tests, are often normal but neutropenia and raised erythrocyte sedimentation rate have been reported. Diagnosis relies on the histological examination of lymph node biopsies. Generally the clinical course is favourable in 3-4 months. Secondary systemic lupus erythomatosus may develop and require regular follow-up. The aetiology of this rare disease is still unclear although certain observations would favour an immunological process. Very rare in France, we report a case of Kikuchi's disease in a pregnant woman and present a review of the literature.
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PMID:[Kikuchi's disease. Apropos of a case. Review of the literature]. 809 19

Systemic lupus erythematosus (SLE) is a rare disease in males. There is evidence that a functional state of hypoandrogenism is important in the pathogenesis of the disease. We analysed the levels of several hormones (follicle stimulating hormone (FSH), luteinizing hormone (LH), testosterone (T), estradiol (E2), free estradiol (FE2) and prolactin (PRL)) in 17 male SLE patients and 17 male healthy controls with similar age distribution. Three lupus patients were excluded from the analysis due to previous cyclophosphamide therapy or pre-puberty. Thus 14 male lupus patients were eligible for the study. Six of the 14 SLE patients (43%) showed at least one abnormal level of FSH, LH or T. There were no abnormalities in these hormones in the 17 controls. This difference was significant (P < 0.01). In five of these 6 male patients (36% of all lupus patients) the hormonal profile was compatible with a functional state of hypoandrogenism (high LH and/or low T). The ratio E2/T (estradiol/testosterone:pmol/nmol) was also significantly higher in the SLE group (average = 6.5; SD 4.3) when compared with that of the control group (average 4.2; SD 1.2; Mann-Whitney rank sum test: P < 0.03). There were no significant differences in E2, FE2 or PRL between lupus patients and controls. We did not confirm the notion that left-handedness is frequent in male lupus as all our patients were right-handed. We found a significantly higher prevalence of sex hormone abnormalities in male lupus patients when compared with healthy controls with a similar age distribution.(ABSTRACT TRUNCATED AT 250 WORDS)
Lupus 1993 Oct
PMID:Systemic lupus erythematosus: sex hormones in male patients. 830 25


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