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

Publish Year: 1395
Type: Journal paper
Language: English
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Document National Code:

JR_IJP-11-3_011

Index date: 23 September 2019

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

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-Iran) in May 2011 because of lower extremities edema and ascites and fever from 1.5 month ago. In physical examinations she had generalized lymphadenopathy, splenomegaly and pleural effusion. In laboratory tests she had pancytopenia, positive ANA and Anti DNA (ds), hypocomplementemia, hypertriglyceridemia and high ferritin level. Gradually she had signs of RPGN and ARDS. The patient had no skin and musculoskeletal signs of SLE and no liver failure nor coagulopathy of MAS. Her lymph node biopsy was reported as Castleman syndrome. Unlike other studies, the patient showed MAS before treatment with cytotoxic for lupus nephritis.

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

Systemic Lupus Erythematosus , Macrophage activating syndrome hemophagocytic lymphohistiosytosis , Castleman syndrome

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

Shamsa Shariatpanahi

Dept. of Internal Medicine, Shahed University, Tehran, Iran

Shahryar Pourfarzam

Dept. of Pathology, Shahed University, Tehran, Iran

Mohammad hosein Gheini

Dept. of Pathology, Shahed University, Tehran, Iran