Nintedanib in chronic fibrosing interstitial lung diseases. A case series.

Progressive pulmonary fibrosis (PPF) can be fatal in non-idiopathic interstitial lung diseases. We report a descriptive series of 13 patients with PPF who received treatment with nintedanib, a multitargeted tyrosine kinase inhibitor with antifibrotic effect. Although the reduced number of patients and the observational nature of a case series prevent us from providing strong evidence, our results suggest that nintedanib could be effective in PPF of various etiologies. Nintedanib could also be useful in specific populations such as patients awaiting lung transplant and elderly patients.

Radiological usual interstitial pneumonia pattern is associated with two-year mortality in patients with idiopathic pulmonary fibrosis.

Introduction: The new diagnostic guidelines for idiopathic pulmonary fibrosis (IPF) did not rule out the possibility of combining the radiological patterns of usual interstitial pneumonia (UIP) and probable UIP, given the similar management and diagnostic capacity. However, the prognostic implications of these patterns have not been fully elucidated, with different studies showing heterogeneous results. We applied the new criteria to a retrospective series of patients with IPF, assessing survival based on radiological patterns, findings, and their extension. Methods: Two thoracic radiologists reviewed high-resolution computed tomography images taken at diagnosis in 146 patients with IPF, describing the radiological findings and patterns. The association of each radiological finding and radiological patterns with two-year mortality was analysed. Results: The two-year mortality rate was 40.2% in IPF patients with an UIP radiological pattern versus 7.1% in those with probable UIP. Compared to the UIP pattern, probable UIP was protective against mortality, even after adjusting for age, sex, pulmonary function, and extent of fibrosis (hazard ratio (HR) 0.23, 95% confidence interval (CI) 0.06-0.99). Receiving antifibrotic treatment was also a protective factor (HR 0.51, 95%CI 0.27-0.98). Honeycombing (HR 3.62, 95%CI 1.27-10.32), an acute exacerbation pattern (HR 4.07, 95%CI 1.84-8.96), and the overall extent of fibrosis (HR 1.04, 95%CI 1.02-1.06) were predictors of mortality. Conclusions: In our series, two-year mortality was higher in patients with IPF who presented a radiological pattern of UIP versus probable UIP on the initial scan. Honeycombing, an acute exacerbation pattern, and a greater overall extent of fibrosis were also predictors of increased mortality. The prognostic differences between the radiological pattern of UIP and probable UIP in our series would support maintaining them as two differentiated patterns.

Mosaic attenuation in non-fibrotic areas as a predictor of non-usual interstitial pneumonia pathologic diagnosis.

The new radiological diagnostic criteria for diagnosing idiopathic pulmonary fibrosis (IPF) seek to optimize the indications for surgical lung biopsy (SLB). We applied the new criteria to a retrospective series of patients with interstitial lung disease (ILD) who underwent SLB in order to analyse the correlation between the radiological findings suggestive of another diagnosis (especially mosaic attenuation and its location with respect to fibrotic areas) and the usual interstitial pneumonia (UIP) pathologic diagnosis. Two thoracic radiologists reviewed the HRCT images of 83 patients with ILD and SLB, describing the radiological findings and patterns based on the new criteria. The association of each radiological finding with radiological patterns and histology was analysed. Mosaic attenuation is highly prevalent in both the UIP and non-UIP pathologic diagnosis and with similar frequency (80.0% vs. 78.6%). However, the presence of significant mosaic attenuation (≥ 3 lobes) only in non-fibrotic areas was observed in 60.7% of non-UIP pathologic diagnosis compared to 20.0% in UIP. This finding was associated with other diagnoses different from IPF, mostly connective tissue disease-associated interstitial lung disease (CTD-ILD) and hypersensitivity pneumonitis (HP). In our series of pathologically confirmed ILD, mosaic attenuation in non-fibrotic areas was a predictor of non-UIP pathologic diagnosis, and was associated with other diagnoses different from UIP, mostly CTD-ILD and HP. If confirmed in larger series, this finding could constitute a valuable tool for improving the interpretation of radiological.

Neumonía organizativa fibrinosa aguda asociada a tratamiento con inmunoterapia (pembrolizumab) / Acute Fibrinous and organizing Pneumonia Associated With Immunotherapy (Pembrolizumab)

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[Translated article] Acute Fibrinous and Organizing Pneumonia Associated With Immunotherapy (Pembrolizumab) / Neumonía organizativa fibrinosa aguda asociada a tratamiento con inmunoterapia (pembrolizumab)

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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.

Sarcoidosis asociada al tratamiento con interferón beta / Sarcoidosis associated with Interferon beta treatment

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Poliangitis microscópica y enfermedad pulmonar intersticial / Microscopic polyangiitis and interstitial lung disease

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Reacción granulomatosa sarcoidosis-like asociada al tratamiento con inmunoterapia (pembrolizumab) / Sarcoidosis-Like Granulomatous Reaction Associated With Pembrolizumab Immunotherapy

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