Systemic lupus erythematosus (SLE) is a multisystem disorder characterised by B-cell hyperactivity with production of multiple autoantibodies. Fever in SLE may be caused by disease exacerbation or by infection. We report a patient of SLE that was later complicated by fever, pancytopenia, and massive splenomegaly. Corticosteroid therapy for SLE might have masked the underlying infection at earlier stage. Despite negative results of rk-39 test and bone marrow biopsy, a very high suspicion for visceral leishmaniasis (VL) led us to go for direct agglutination test (DAT) and polymerase chain reaction (PCR) for leishmanial antigen that revealed positive results. Moreover, significant improvement in clinical and biochemical parameters was noted on starting the patient on antileishmanial therapy.

Sunny Garg

Mousumi Kundu

Amit Nandan Dhar Dwivedi

Lalit Prashant Meena


							Neeraj Varyani

Asif Iqbal

Kamlakar Tripathi

Visceral Leishmaniasis or Systemic Lupus Erythematosus Flare?

We present here a novel case of an atypical Omenn syndrome (OS) phenotype due to mutations in the ADA gene encoding adenosine deaminase. This case is noteworthy for a significant increase in circulating CD56brightCD16- cytokine-producing NK cells after treatment with steroids for skin rash.

Avni Y. Joshi

Erin K. Ham

Neel B. Shah

Xiangyang Dong

Shakila P. Khan

Roshini S. Abraham

Atypical Omenn Syndrome due to Adenosine Deaminase Deficiency

Antiphospholipid syndrome is a disorder presenting with arterial or venous thrombus and a history of fetal loss. Early diagnosis and adequate treatment is important to prevent multiple organ failures. Here, we described a woman with a two-year history of systemic lupus erythematosus with severe nephrotic syndrome, manifested multiple thrombi over the portal vein and the inferior vena cava, combined with acute renal infarction. The patient underwent splenectomy 10 months ago. Initially, she received anticoagulant treatment and low-dose glucocorticoid, but multiple organ failure progressed. After emergency plasma exchange followed by glucocorticoid pulse therapy, the patient recovered.

Deng-Ho Yang

Multiple Thromboses in a Patient with Systemic Lupus Erythematosus after Splenectomy

Toxic epidermal necrolysis is the most severe form of drug-induced skin reaction and includes denudation of &#62;30% of total body surface area. The mechanism of disease is not completely understood, but immunologic mechanisms, cytotoxic reactions, and delayed hypersensitivity seem to be involved. We report a case of cephazolin-induced toxic epidermal necrolysis treated with intravenous immunoglobulin and N-acetylcysteine with excellent response.

Carlos Saavedra

Paola Cárdenas

Héctor Castellanos

Kateir Contreras

J. R. Castro

Cephazolin-Induced Toxic Epidermal Necrolysis Treated with Intravenous Immunoglobulin and N-Acetylcysteine

Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder with involvement of multiple organs. Various forms of serositis, including pleural effusion, pericardial effusion, and ascites, may be found during the course of SLE. Peritoneal involvement by ascites is common in the initial presentation of SLE. However, chylous ascites is uncommon in SLE patients. Here, we describe a 93-year-old female with initial presentation of chylous ascites during SLE flares. Marked distention and an ovoid shape of the abdomen were observed. Shifting dullness and central tympanic sounds were found on percussion. Rales were heard in bilateral breathing sounds, multiple oral ulcers appeared in the oral cavity, and chest images showed bilateral pleural effusion. Abdominal sonography revealed moderate ascites and pleural effusion. Neither organisms nor malignant cells were revealed in the culture or cytology of ascites and pleural effusion. The diagnosis of SLE was arrived at by positive antinuclear antibody (ANA), discoid rash, oral ulcers, serositis (pleural effusion and ascites), and proteinuria. The patient received intravenous methylprednisolone 250 mg/day for three days. The pleural effusion resolved dramatically after steroid therapy and abdominal distention related to ascites formation subsided obviously.

Guan-Liang Chen

Wen-Hsiu Hsu

Chylous Ascites and Pleural Transudate: Rare Presentations in Systemic Lupus Erythematosus in Old Age

K&#252;ttner tumor is a chronic inflammatory disease that presents with a firm swelling of the submandibular gland and often mimics a neoplasm. Recently evidence suggests that K&#252;ttner tumor may be a type of disorder characterized by IgG4-related inflammations. Herein, we report 3 cases of submandibular gland swellings with severe fibrosis, inflammation with marked lymphoplasmacytic infiltration; this pathology mimics clinical manifestation of a malignant tumor in 18-fluorodeoxyglucose positron emission tomography (FDG-PET) findings.

Masayuki Ishida

Hiroaki Fushiki

Yukio Watanabe

An IgG4-Related Salivary Gland Disorder: A Case Series Presenting with a Different Clinical Setting

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Case Reports in Immunology publishes case reports and case series related to allergies, immunodeficiencies, autoimmune diseases, immune disorders, cancer immunology and transplantation immunology.

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Case Reports in Immunology publishes case reports and case series related to allergies, immunodeficiencies, autoimmune diseases, immune disorders, cancer immunology and transplantation immunology.

Case Reports in Immunology