Doença sistêmica relacionada à IgG4 com linfopenia: relato de caso e breve revisão de literatura / IgG4-related systemic disease associated with lymphopenia: case report and brief literature review

A doença sistêmica relacionada à IgG4 é uma doença emergente, recentemente descrita, caracterizada clinicamente por aumento parcial ou total de um órgão, e, por isso, com amplo espectro de manifestações clínicas. Esta é uma doença sistêmica fibroinflamatória, patologicamente provocada pela infiltração de plasmoblastos IgG4 positivos que levam à inflamação eosinofílica do tecido e, consequentemente, fibrose estoriforme. Quando o diagnóstico é precoce, a melhora clínica e resposta sustentada com corticosteroides sistêmicos é impressionante. O diagnóstico é baseado em critérios patológicos, mas, recentemente, alguns trabalhos têm descrito que plasmoblastos no soro podem servir como um fator independente para auxiliar no diagnóstico da doença. Este artigo descreve uma apresentação atípica da doença relacionada à IgG4, em um paciente linfopênico com medição inconclusiva de plasmoblastos no soro.

IgG4-related systemic disease is a recently described, emerging condition, clinically characterized by partial or total enlargement of an organ, with a broad spectrum of clinical manifestations. It is a systemic fibroinflammatory condition caused by the infiltration of IgG4-positive plasmablasts that lead to eosinophilic inflammation of tissues and consequently storiform fibrosis. When diagnosis is early, clinical improvement and maintained response achieved with systemic corticosteroids is impressive. Diagnosis is based on pathological criteria, but recent papers have described that serum plasmablasts may serve as an independent factor to aid in diagnosis. This paper describes an atypical presentation of IgG4-related disease in a lymphopenic patient with inconclusive serum plasmablast measurement.

Clinical Update on Autoimmune Pancreatitis / 대한내과학회지

Autoimmune pancreatitis (AIP) is a unique type of chronic pancreatitis in which pathogenesis involves autoimmune mechanism. During the past decade, several different diagnostic criteria for AIP have been reported from various countries. A set of international consensus diagnostic criteria and algorithm for AIP has recently been proposed by a consensus of expert opinion. The goal of the international consensus diagnostic criteria and algorithm for AIP is to develop criteria that can be applied worldwide, taking into consideration the marked differences in clinical practice patterns between countries. They also aim at diagnosing AIP nonsurgically and avoiding misdiagnosis of pancreatobiliary malignancies as AIP. This review discusses the recent advances in the diagnosis and management of AIP based on the international consensus diagnostic criteria. We also suggest modified algorithm for diagnosing AIP for Korean practice.

Retroperitoneal fibrosis:a clinical and outcome analysis of 33 cases and review of literature / 中华风湿病学杂志

Objective To investigate the clinical features and outcome of retroperitoneal fibrosis (RPF).Methods Thirty-three cases of RPF treated in the Department of Rheumatology and Immunology were analyzed retrospectively,including the clinical characteristics and laboratory data.Results RPF occurred predominantly in elderly men with untypical clinical manifestations such as back pain,abdominal pain and lower limb edema.In laboratory tests,the inflammatory parameters such as erythrocyte sedimentation rate and C-reactive protein increased significantly,and renal function failure was frequent finding in patients with urethral obstruction.All patients had retroperitoneal soft tissue image or urethral obstruction on computed tomography or magnetic resonance imaging,four of which had histological diagnosis of idiopathic RPF.Twenty-three patients received surgical interventions.Eighteen patients received medical treatment alone including corticosteroids,immunosuppressants,and tamoxifen.Nine patients received corticosteroids after surgical intervention.Surgery and medical treatments are effective for idiopathic RPF.Conclusion Image examination is valuable in diagnosis and differential diagnosis of PRF.Pathological diagnosis is the golden standard.Long-term low dose corticosteroids and immunosuppressants may prevent relapse of idiopathic RPF.