Medicina clinica · 2021
Granulomatous lymphocytic interstitial lung disease: description of a series of 9 cases.
Клинический случайСтатьяНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Ruiz-Alcaraz Sandra, Gayá García-Manso Ignacio, Marco-De La Calle Francisco Manuel, García-Mullor María Del Mar, López-Brull Helena, García-Sevila Raquel
Аннотация
INTRODUCTION: Granulomatous-lymphocytic interstitial lung disease (GLILD) is one of the most serious non-infectious complications in patients with common variable immunodeficiency (CVID). Its diagnosis and treatment are challenging.
OBJECTIVE: To analyse the characteristics of Hospital General Universitario de Alicante patients with CVID and GLILD.
MATERIAL AND METHODS: Descriptive study of patients with CVID and GLILD diagnosed from 2000 to 2020.
RESULTS: Of the 42 patients with CVID, 9 had GLILD (21%). Mean age at diagnosis of 39 years. Sixty-six percent of the CVID was type MB0. Fifty-five percent had decreased BLs. There was a decrease in DLCO by 89%. Surgical lung biopsy (SLB) was performed in 78%. The most frequent extrapulmonary manifestation was adenopathy (78%). One patient had a heterozygous pathological mutation in the CTLA4 gene. Of the patients, 67% received combined corticosteroid treatment with Rituximab.
CONCLUSIONS: GLILD is a rare complication of CVID whose diagnosis and treatment are a challenge. Its diagnosis requires a high index of suspicion, therefore a multidisciplinary diagnostic approach and combined treatment could provide a good result in the adult population.
OBJECTIVE: To analyse the characteristics of Hospital General Universitario de Alicante patients with CVID and GLILD.
MATERIAL AND METHODS: Descriptive study of patients with CVID and GLILD diagnosed from 2000 to 2020.
RESULTS: Of the 42 patients with CVID, 9 had GLILD (21%). Mean age at diagnosis of 39 years. Sixty-six percent of the CVID was type MB0. Fifty-five percent had decreased BLs. There was a decrease in DLCO by 89%. Surgical lung biopsy (SLB) was performed in 78%. The most frequent extrapulmonary manifestation was adenopathy (78%). One patient had a heterozygous pathological mutation in the CTLA4 gene. Of the patients, 67% received combined corticosteroid treatment with Rituximab.
CONCLUSIONS: GLILD is a rare complication of CVID whose diagnosis and treatment are a challenge. Its diagnosis requires a high index of suspicion, therefore a multidisciplinary diagnostic approach and combined treatment could provide a good result in the adult population.