Behçet’s syndrome is a chronic multisystem vasculitis characterized by mucocutaneous, articular, neurological, gastrointestinal and ophthalmological lesions. Behcet’s syndrome is a disease of uncertain etiology. It may be due to immune dysregulation including circulating immune complex, autoimmune cytokines and heat shock proteins are major factor in the pathogenesis of Behcet’s syndrome. Patient’s positive pathergy test suggests a diagnosis of Behcet’s disease. Corticosteroids and immunosupressive drugs are the first line of treatment. This is a case report of juvenile Behcet’s syndrome in a thirteen year old girl associated with arthritis.

BACKGROUND: Autoimmune hemolytic anemia (AIHA) is a less recognized, potentially fatal condition. There is a scarcity of data on clinicoserological characteristics and response to therapy concerning this disease from South India. METHODS: Data for 33 patients with primary AIHA recorded from July 2009 to June 2015 were retrospectively analyzed for clinical presentation, response to frontline therapy, durability of response, time to next treatment (TTNT), and response to second-line agents. RESULTS: The median follow-up period was 50 months. Among 33 patients, 48% of the cases were warm autoimmune hemolytic anemia (WAIHA), 46% were cold agglutinin disease (CAD), and 6% were atypical. Three-fourth of patients had severe anemia (<8 g/dL hemoglobin [Hb]) at onset; younger patients (age <40 yr) had more severe anemia. All of the patients who required treatment received oral prednisolone at 1.5 mg/kg/d as a frontline therapy, and the response rate was 90% (62% complete response [CR] and 28% partial response [PR]). The overall response to corticosteroids in WAIHA and CAD was 87% and 92%, respectively. The median corticosteroid duration was 14 months, and 50% of the patients required second-line agents. Fourteen patients received azathioprine as a second-line agent, and 11 of these patients responded well, with half of them not requiring a third agent. Four patients developed severe infections (pneumonia, sepsis, and soft tissue abscess) and two had life-threatening venous thrombosis. One case of death was recorded. CONCLUSION: AIHA is a heterogeneous disease that requires care by physicians experienced in treating these patients.
Adrenal Cortex Hormones ; Anemia ; Anemia, Hemolytic, Autoimmune* ; Azathioprine ; Follow-Up Studies ; Humans ; India* ; Prednisolone ; Retrospective Studies ; Sepsis ; Venous Thrombosis