Macrophage activation syndrome in a patient with systemic lupus erythematosus

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Association of Macrophage Activating Syndrome with Castleman’s Syndrome in Systemic Lupus Erythematosus

Macrophage Activating Syndrome (MAS) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still's disease, polyarteritis nodosa. It is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. A 27 yr. old female patient was admitted in shahid Mostafa Khomeini Hospital (Tehran-Ira...

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Refractory Angioedema in a Patient with Systemic Lupus Erythematosus

Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported.In lupus patients, angioedema may be the result of an ac...

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Macrophage activation syndrome in a patient with juvenile systemic lupus erythematosus

Introduction Macrophage activation syndrome (MAS) is a rare but life threatening complication of infectious, neoplastic and rheumatic diseases. Clinically, patients usually have high fever, hepatosplenomegaly, lymphadenopathy and neurologic symptoms. In laboratory results pathognomonic are pancytopenia, decline in the value of sedimentation rate (ESR), increased levels of ferritin and coagulati...

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Macrophage activation syndrome revealing systemic lupus erythematosus: about 4 cases

Results We report on four girls, aged of 13, 13, 10 and 7 years old, initially admitted at our university hospital for prolonged fever. Main infectious disease causes were ruled out and a diagnosis of MAS was made according to the criteria of HLH 2004. During the follow up, all patients presented with SLE symptoms and fulfilled the modified ARA criteria. Glomerular nephropathy was clinically di...

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Pyoderma gangrenosum in a patient with antiphospholipid antibody negative systemic lupus erythematosus: A case report

In any description of leg ulcers in systemic lupus erythematosus (SLE), pyoderma gangrenosum (PG) earns a mention at least for its being quite rare in such patients. The causative role of aPL (antiphospholipid antibody) in dermatological manifestations of SLE is undermined by the occurrence of PG in aPL negative SLE patients. To the best of our knowledge, there are only two reports of PG in aPL...

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ژورنال

عنوان ژورنال: Polish Archives of Internal Medicine

سال: 2019

ISSN: 1897-9483

DOI: 10.20452/pamw.14953