Transplante pulmonar em pacientes com fibrose pulmonar familiar / Lung transplant in patients with familial pulmonary fibrosis

RESUMO Objetivo A fibrose pulmonar familiar (FPF) é definida como uma doença pulmonar intersticial idiopática que afeta dois ou mais membros da mesma família. Nesses pacientes, os resultados têm sido insatisfatórios, apresentando alto risco de morte e disfunção crônica do enxerto pulmonar (CLAD) após o transplante de pulmão. O objetivo do presente estudo foi comparar o resultado de curto e longo prazo do transplante de pulmão em pacientes com FPF e pacientes transplantados por outras doenças pulmonares intersticiais. Métodos Foram coletados retrospectivamente dados clínicos pré e pós-transplante de 83 pacientes com fibrose pulmonar submetidos a transplante de pulmão. Os pacientes foram divididos em aqueles com fibrose pulmonar familiar (n = 9, grupo FPF) e aqueles com fibrose pulmonar não familiar (n = 74, grupo controle). Resultados O grupo FPF foi composto de quatro mulheres e cinco homens, sendo 44,5% ex-fumantes. A maioria apresentou tomografia computadorizada e evidência patológica de pneumonia intersticial usual. Os pacientes com FPF tiveram níveis significativamente menores de hemoglobina e hematócrito. Não foram observadas outras diferenças nas características pré e pós-transplante em relação ao grupo controle. A evolução clínica pós-operatória foi semelhante nos dois grupos. Não foi constatada diferença significativa na sobrevida de um ano livre de CLAD e na sobrevida em geral. Conclusão A evolução pós-transplante de pacientes com FPF foi semelhante à dos pacientes com fibrose pulmonar não familiar, embora mais pacientes com FPF tivessem anemia pré-transplante. O resultado a curto e longo prazo foi comparável em ambos os grupos. O transplante de pulmão provou ser uma opção válida para pacientes com FPF, assim como para pacientes com outros tipos de fibrose pulmonar.

ABSTRACT Objective Familial pulmonary fibrosis (FPF) is defined as an idiopathic interstitial lung disease affecting two or more members of the same family; poor outcome with high risk of death and chronic lung allograft dysfunction (CLAD) after lung transplant has been reported in these patients. The present study aimed to compare the short- and long-term outcome of lung transplants in patients with FPF and patients transplanted because of other interstitial lung diseases. Method Clinical pre- and post-transplant data from 83 consecutive patients with pulmonary fibrosis who underwent lung transplant at our centre were collected retrospectively. Patients were divided into those with familial (n=9 FPF group) and those with non-familial pulmonary fibrosis (n=74 controls). Results The FPF group was composed of 4 females and 5 males; 44.5% were ex-smokers. The majority presented their CT scan and pathology evidence of usual interstitial pneumonia. Patients with FPF had significantly lower pre-transplant levels of haemoglobin and haematocrit. No other differences in pre- and post-transplant characteristics were observed concerning controls. The clinical post-operative course was similar in the two groups. No significant difference in one-year CLAD-free survival and overall survival was observed. Conclusion The post-transplant course of patients with FPF was similar to patients with non-familial pulmonary fibrosis, although more patients with FPF had pre-transplant anaemia. Short- and long-term outcome was comparable in both groups. Lung transplant proved to be a valid option for patients with FPF as it was for patients with other types of pulmonary fibrosis.

Pleuroparenchymal fibroelastosis: report of two cases in Brazil / Fibroelastose pleuroparenquimatosa: relato de dois casos no Brasil

ABSTRACT Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease. It can be idiopathic or associated with any one of various conditions. To our knowledge, this is the first report of two cases of PPFE in Brazil. Our first patient presented with pleural and subpleural fibrosis in the upper lobes; a spiculated nodule in the left upper lobe; and a mild reticular pattern in the lower lobes. Surgical lung biopsy revealed PPFE in the upper lobes, including the nodule, and unclassified interstitial pneumonia in the left lower lobe. Our second patient had a history of exposure to domestic birds, indicating a risk of hypersensitivity pneumonitis, and presented with advanced lung disease, predominantly in the upper lobes, together with subpleural fibrosis.That patient underwent lung transplantation. In the explant specimen, PPFE and granulomas were identified, suggesting hypersensitivity pneumonitis as an associated cause.

RESUMO A fibroelastose pleuroparenquimatosa (FEPP) é uma doença pulmonar rara, podendo ser idiopática ou associada a diversas condições. Pelo que sabemos, este é o primeiro relato de dois casos de FEPP no Brasil. Nosso primeiro paciente apresentava fibrose pleural e subpleural nos lobos superiores, um nódulo espiculado no lobo superior esquerdo e um padrão reticular discreto nos lobos inferiores. A biópsia pulmonar cirúrgica demonstrou FEPP nos lobos superiores, incluindo no nódulo, e pneumonia intersticial não classificada no lobo inferior esquerdo. Nosso segundo paciente tinha história de exposição a aves domésticas, indicando um risco de pneumonite de hipersensibilidade, e doença pulmonar avançada predominando em lobos superiores, com fibrose subpleural. Esse paciente foi submetido a transplante pulmonar. No espécime do explante, FEPP e granulomas foram identificados, sugerindo pneumonite de hipersensibilidade como causa associada.

Congenital malformation of lung parenchyma: 15 years experience in a thoracic surgical unit.

Objective. To review the surgical management of congenital malformations of lung parenchyma in a thoracic surgery unit over a period of 15 years. Methods. We carried out a retrospective analysis of records of all patients who had surgery for congenital malformations of lung parenchyma between 1995 and 2010. Results. Forty-five patients underwent surgery for congenital lung lesions out of 3735 thoracotomies performed during the study period. The lesions included 29 lung sequestrations, 12 bronchogenic cysts, 3 congenital lobar emphysema and one congenital cystic adenomatoid malformation. Only 26 (26%) cases were diagnosed preoperatively. Twenty-eight (62.2%) patients underwent lobectomy, 5 (11.1%) patients had pneumonectomy, and 10 (22.2%) patients had removal of cyst while 2 (0.45%) patients had lung resection with repair of the oesophageal connection. No mortality was recorded. One patient had post-operative complication of oesophageal fistula which was successfully managed conservatively. The follow-up was between 8 months to 14 years. All patients were asymptomatic and had no physical limitations during the follow-up. Conclusions. Surgery is curative and produces good long-term result in patients with congenital malformations of lung parenchyma. It should be offered to patients as a therapeutic option where indicated and feasible.

Video assisted thoracoscopic surgery [VATS] lung biopsy for non-responsive patients of interstitial lung disease [ILD]

To study the results of VATS [Video Assisted Thoracoscopic Surgery] Lung Biopsy in Interstitial Lung Disease [ILD] case series. Department of Thoracic Surgery, CMH Rawalpindi from Jan 2009 to Mar 2010. All those patients of ILD [Interstitial Lung Disease] were included who failed to improve after a period of observation including those on empiric therapy, all patients in which BAL [Bronchoalveolar Lavage] and TBLB [Transbronchial Lung biopsy] was inconclusive and all those patients fit to undergo thoracoscopic surgery. Patients excluded were elderly patients, those with systemic disorders frequently associated with ILD and those with known neoplasm likely to have lymphangitic dissemination. We used three thoracoscopic ports, all biopsies were sent for histopathology examination, single chest tube was placed in all cases and it was removed once the airleak ceased. All complications encountered were noted. Fourteen patients were registered during study period. Biopsy showed that 9 had ILD, and 5 did not have ILD. Only complication was a prolonged airleak for 2 days in 2 patients. No mortality was encountered. Thoracoscopic surgical biopsy can be accomplished safely in most cases where there is a diagnostic dilema for interstitial lung disease

Biopsia en enfermedad intersticial pulmonar / Lung biopsy for the diagnosis of interstitial lung disease

El objetivo del presente estudio fue determinar el rédito diagnóstico y los factores asociados a mayor morbimortalidad en la biopsia quirúrgica de pulmón en pacientes con enfermedad intersticial difusa. Se analizaron en forma retrospectiva los registros clínicos de 71 pacientes. Se registraron complicaciones en 16 pacientes (22.5%). La mortalidad operatoria fue 11.2%. Los pacientes en quienes la biopsia se realizó por videotoracoscopia (n = 52) y por toracotomía (n = 17) tuvieron la misma duración de estadía en terapia intensiva y de estadía hospitalaria. La tasa de complicaciones (22.2% vs. 21.0%, p = 1.0000) y la mortalidad (9.2 vs. 15.7%, p = 0.2738) no fueron diferentes. Ocho pacientes murieron dentro de los 30 días. La prevalencia de inmunosupresión (4/8 vs. 9/63, p = 0.0325) fue significativamente superior en el grupo de pacientes fallecidos. Estos pacientes tuvieron valores preoperatorios más elevados de urea (50 ± 20.1 mg/dl vs. 31.2 ± 10.3 mg/ dl, p = 0.0013) y menores valores de saturación de O2: 82.7 ± 14.8% vs. 92.8 ± 3.4%, p = 0.0009. En los 11 pacientes con iniciación aguda la mortalidad fue significativamente más elevada (36.3% vs. 7.1%, p = 0.0223). La biopsia aportó un diagnóstico específico en 100% de los pacientes y cambió la estrategia terapéutica en 66.7%. En conclusión, la biopsia de pulmón por vía toracoscópica es un procedimiento útil y seguro en los pacientes con enfermedad intersticial difusa del pulmón. Sin embargo, en el grupo de pacientes inmunocomprometidos, con cuadros de presentación aguda y con insuficiencia respiratoria preoperatoria, la mortalidad es elevada y deben balancearse muy críticamente los riesgos contra los beneficios en ese grupo de enfermos.

The objective of this study was to determine the morbidity, mortality and diagnostic yield of video assisted thoracoscopy (VATS) and thoracotomy lung biopsy in interstitial lung disease (ILD). Clinical records of 71 patients were retrospectively analyzed. There was no difference in mean hospital stay, intensive care unit stay and duration of chest tube drainage in patients with VATS (n = 52) compared with those undergoing open thoracotomy (n = 17). Complications rate (22.2% vs. 21.0%, p = 1.0000) and operating mortality (9.2 vs. 15.7%, p = 0.2738) were also similar. Overall, complications occurred in 16 patients (22.5%). Thirty-day mortality rate was 11.2% (n = 8). Prevalence of immunosupression (4/8 vs. 9/63, p = 0.0325) was significantly higher in the group of patients who died. No surviving patients had higher values of plasmatic urea (50 ± 20.1 mg/dl vs. 31.2 ± 10.3 mg/dl, p = 0.0013) or lower values of preoperative oxygen saturation (SaO2): 82.7 ± 14.8% vs. 92.8 ± 3.4%, (p = 0.0009). Eleven patients had an acute illness. Those patients did not show a higher complication rate (4/11 vs. 10/45, p = 0.4390) but mortality was significantly higher (4/11, 36.3% vs. 3/45, 7.1%, p = 0.0223). Biopsy allowed a specific histologic diagnosis in 100% of patients and changed therapy in 66.7%. We conclude that surgical lung biopsy is a safe and useful procedure in patients with ILD. However the higher mortality rate in patients with acute symptoms, immunocompromise, or in respiratory failure must be balanced against potential benefits of altering treatment decisions.

Estudio comparativo entre biopsia pulmonar a cielo abierto y toracoscópica, como método diagnóstico en neumopatía intersticial difusa / Comparative study between open and thorascopic pulmonar biopsy, as diagnostic method in diffuse interstitial pneumophaty

Se estudiaron 15 pacientes con diagnóstico clínico, funcional y radiológico de neumopatía intersticial difusa, 11 mujeres y 4 hombres de 15 a 40 años. A todos los pacienes se les praticó en el mismo tiempo operatorio, toracoscopia y biopsia pulmonar a cielo abierto (pequeña incisión submamaria). El tejido pulmonar obtenido por biopsia pulmonar se envió a estudio histológico inmediatamente. La biopsia por toracoscopia se colocó en formol y se guardó por dos meses con etiquetas sólo conocidas por un investigador y no por el patólogo. Cuando el patólogo estudió la biopsia por toracoscopia lo hizó en forma ciega e independiente. Se concluyó que la toracoscopia y la biopsia pulmonar a cielo abierto brindan la misma sensibilidad y especificidad