Adolescente con fibroelastosis pleuroparenquimatosa idiopatica: A propòsito de un caso / An adolescent with idiopathic pleuroparenchymal fibroelastosis: Case report

La fibroelastosis pleuroparenquimatosa idiopàtica (FEPPI) es un trastorno raro incluido recientemente en las neumonías intersticiales idiopáticas según la actualización de la clasificación de la Sociedad Torácica Estadounidense (American Thoracic Society) y la Sociedad Respiratoria Europea (European Respiratory Society). La FEPPI se caracteriza por fibrosis parenquimatosa pleural y subpleural que produce reducción del volumen, sobre todo en los lóbulos pulmonares superiores. La edad al momento de la aparición varía, aunque la FEPPI ocurre principalmente entre los 30 y los 50 años de edad. En este artículo, presentamos el caso de un paciente de 16 años con antecedentes de disnea de esfuerzo, tos seca y pérdida de peso en los últimos dos años. Durante el examen físico, con la auscultación se detectó disminución de los ruidos respiratorios en los lóbulos superiores. En la radiografía de tórax se observó engrosamiento pleural apical y volumen reducido. En la tomografia computarizada (TC) del tórax se observaron densidades en vidrio esmerilado y bronquiectasia tubular predominantemente en ambos lóbulos superiores, con engrosamiento septal interlobulillar de la pleura y adenopatía mediastínica. Se realizó una biopsia pulmonar por toracoscopia y en el examen histológico se observaron fibrosis subpleural y tinción de las fibras elásticas que demostraba depósito de fibras elásticas en el área subpleural y el parénquima pulmonar adyacente, lo que sugería FEPPI. Hasta donde sabemos, este es el primer caso durante la niñez. Por lo tanto, los pediatras deben estar atentos a esta enfermedad para realizar un diagnóstico y tratamiento adecuados.

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disorder recently included in rare idiopathic interstitial pneumonias according to the updated American Thoracic Society/European Respiratory Society classification. IPPFE is characterized by pleural and subpleural parenchymal fibrosis causing volume loss predominantly in the upper lung lobes. Age of onset is variable, IPPFE mainly occurs in third and fourth decades. We present a 16 year old patient with a 2-year history of exertional dyspnea, nonproductive cough and weight loss. On physical examination, auscultation revealed diminished breath sounds on the upper lobes.Chest radiograph showed apical pleural thickening and volume loss. Computerized tomographic scan (CT) of chest revealed ground glass densities and tubular bronchiectasis predominantly in upper lobes bilaterally, with interlobular septal thickening of the pleura and enlarged mediastinal lymph nodes. Thoracoscopic lung biopsy was performed and histological evaluation showed subpleural fibrosis and elastic staining demonstrated fragmented elastic fiber deposition in the subpleural area and adjacent pulmonary parenchyma suggesting IPPFE. To our knowledge this is the first case in childhood. Therefore, pediatricians should be aware of this disease for the diagnosis and appropriate management.

Interleukin-13 and Its Receptors in Idiopathic Interstitial Pneumonia: Clinical Implications for Lung Function

Idiopathic interstitial pneumonia (IIP) is characterized by varying degrees of interstitial fibrosis. IL-13 and IL-4 are strong inducers of tissue fibrosis, whereas IFN-gamma has antifibrotic potential. However, the roles of these substances in IIP remain unknown. IL-13, IL-4, and IFN-gamma were measured in the BAL fluid of 16 idiopathic pulmonary fibrosis (IPF) patients, 10 nonspecific interstitial pneumonia (NSIP) patients, and 8 normal controls. The expression of IL-13 and IL-13Ralpha1/alpha2 in lung tissues was analyzed using ELISA and immunohistochemistry. IL-13 levels were significantly higher in IPF patients than the others (P<0.05). IL-4 levels were higher in both IPF and NSIP patients than in normal controls (P<0.05), and IFN-gamma levels were lower in NSIP patients than in normal controls (P=0.047). IL-13 levels correlated inversely with FVC% (r=-0.47, P=0.043) and DLCO% (r=-0.58, P=0.014) in IPF and NSIP patients. IL-13 was strongly expressed in the smooth muscle, bronchial epithelium, alveolar macrophages and endothelium of IPF patients. IL-13Ralpha1, rather than IL-13Ralpha2, was strongly expressed in the smooth muscle, bronchial epithelium, and endothelium of IPF patients. IL-13 and its receptors may contribute to the pathogenesis of fibrosis in IIP and appear to be related to the severity of the disease.