Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 57-year-old man was admitted to our clinic with complaints of proximal myalgia in extremities. He was diagnosed as late-onset SLE based on the findings of pleuritis, pericarditis, arthritis and antibodies to DNA and cardiolipin. Aggregation of the platelets and the decreased counts of platelets were observed when EDTA was used as anticoagulant for the blood tests. However, the platelet aggregation was not noted with normal counts of platelets when Heparin-Theophylline was used as anticoagulant. From this observation, EDTA-dependent pseudothrombocytopenia was diagnosed and IgM class of EDTA-dependent anti-platelet antibody was detected by means of flow cytometry. Administration of prednisolone at 40mg/day reduced the symptoms and EDTA-dependent pseudothrombocytopenia, and EDTA-dependent anti-platelet antibody disappeared. His clinical course suggested that EDTA-dependent pseudothrombocytopenia was closely associated with the disease activity of SLE.
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PMID:[A case of late-onset SLE complicated with EDTA-dependent pseudothrombocytopenia]. 144 83

Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965-2015). These 80 cases, together with our 9 patients, form the basis of the present analysis.The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases.An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful.There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength.The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild to moderate improvement on pulmonary function tests. The mortality rate was very low.
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PMID:Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature. 2753 1