Enfermedad pulmonar intersticial linfocítica granulomatosa: descripción de una serie de 9 casos / Granulomatous lymphocytic interstitial lung disease: description of a series of 9 cases

Introducción: La enfermedad pulmonar intersticial linfocítica granulomatosa (GLILD) es una de las complicaciones no infecciosas más graves de los pacientes con inmunodeficiencia común variable (IDCV). Su diagnóstico y tratamiento suponen un reto.ObjetivoAnalizar las características de los pacientes con IDCV y GLILD del Hospital General Universitario de Alicante.Material y métodosEstudio descriptivo de los pacientes con IDCV y GLILD diagnosticados desde 2000 a 2020.ResultadosDe los 42 pacientes con IDCV 9 presentaban GLILD (21%). La edad media al diagnóstico fue de 39 años. El 66% de IDCV fue de tipo MB0. El 55% tenía los linfocitos LB disminuidos. Se observó una disminución de la capacidad de transferencia del monóxido de carbono en un 89%. La biopsia pulmonar quirúrgica (BPQ) se realizó en el 78%. La manifestación extrapulmonar más frecuente fue adenopatías (78%). Una paciente presentó mutación patológica en heterocigosis en el gen CTLA4. El 67% de los pacientes recibió tratamiento combinado de corticoides con rituximab.ConclusionesLa GLILD es una complicación infrecuente de las IDCV cuyo diagnóstico y tratamiento es un reto. Su diagnóstico requiere un alto índice de sospecha, por lo que el enfoque diagnóstico multidisciplinar y el tratamiento combinado podrían proporcionar un buen resultado en la población adulta. (AU)

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.ObjectiveTo analyse the characteristics of Hospital General Universitario de Alicante patients with CVID and GLILD.Material and methodsDescriptive study of patients with CVID and GLILD diagnosed from 2000 to 2020.ResultsOf 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.ConclusionsGLILD 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. (AU)

[Granulomatous lymphocytic interstitial lung disease: description of a series of 9 cases]. / Enfermedad pulmonar intersticial linfocítica granulomatosa: descripción de una serie de 9 casos.

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.