Histological and clinical characteristics of cases of immunoglobulin G4-related disease in a tertiary hospital, in Cali, Colombia

• Published in: Revista española de patología Vol. 51; no. 4; pp. 224 - 231
• Main Authors: Silva, Nhora, Calvache, Natalia, Muñoz, Noelia, Londoño, Ricardo, Pérez, Bladimir, Pacheco, Robinson
• Format: Journal Article
• Language: Spanish
• Published: Spain 01.10.2018
• Subjects: Colombia; Patrones histológicos; Diagnóstico; IgG4-related disease; Células plasmáticas; Histological patterns; Plasma cells; Enfermedad relacionada con IgG4; IgG4; Diagnosis
• ISSN: 1988-561X
• DOI: 10.1016/j.patol.2018.01.004

Immunoglobulin G4 (IgG4)-related disease has been described in the last decade. It is a fibro-inflammatory condition capable of affecting almost every organ and diagnosis requires both clinical and paraclinical confirmation. We present the largest study to date in Colombia. To describe the clinical and histopathological characteristics of patients diagnosed with IgG4-related disease at the Fundación Valle del Lili. Observational-descriptive retrospective study. The clinical and pathological records of patients diagnosed with IgG4-related disease at the Fundación Valle del Lili were reviewed and a descriptive statistical analysis made. From 2013-2016, 16 patients were diagnosed. Median age was 44 years (RIC 30-58) and 10 (62.5%) were women. The most common clinical presentation was a combination of a tumefactive mass, constitutional symptoms and site-related symptoms (43.8%) (n=7). No preference for any organ was seen. Histopathology revealed all cases had dense lymphoplasmacytic infiltrate and storiform-type fibrosis; 75% also had obliterative phlebitis. In all cases≥10 cells/HPF of IgG4+ were found and 81% had a ratio of IgG4+/IgG+>50%. IgG4-related disease appears to be underdiagnosed, probably due to its broad clinical spectrum as well as a low index of awareness among clinicians. We recommend that, when dense lymphoplasmacytic infiltrates, storiform-type fibrosis or obliterative phlebitis are found, immunohistochemistry for IgG and IgG4should be requested. Positive results then must be correlated with complementary studies to confirm the disease.