Pragmatic Real-world Approach to Treat Diffuse Parenchymal Lung Disease in Logistically Constraint Situations: A Proposition

Understanding the etiology and the tempo of progression with inexorable and self-perpetuating fibrosis [identification of progressive-fibrotic (PF) phenotype of interstitial lung disease (ILD) (PF-ILD)] can help to decide the treatment of diffuse parenchymal lung disease (DPLD) in the real-world practice. An evidence-supported pragmatic approach has been forwarded for such circumstances.

Six-Minute Walk Test Outcomes in Patients with Diffuse Parenchymal Lung Disease

The 6-minute walk test (6MWT) is used to measure exercise capacity and assess prognosis in diffuse parenchymal lung disease (DPLD). Although the 6MWT is usually considered to be a test of submaximal exercise capacity in DPLD, the physiological load imposed by this test is not well described and 6MWT outcomes are poorly understood. This study aimed to compare cardiorespiratory responses to 6MWT in people with DPLD. Methods: An observational crosssectional study was carried out in a single tertiary care center in northern India which included 50 consecutive subjects (age >12 years) who had diffuse parenchymal lung diseases. In this study, the clinical, radiological and histological data subjects was collected. 6MWT was done of all the patients and results tabulated. Result: Desaturation on 6MWT (SPo2 <88% or fall of 4% from baseline) were seen in total of 33(66%) patients with most cases being IPF in which 17 patients (89.47%) out 19 cases showed desaturation. This was followed by NSIP with 4(67%) of of 6 cases and CTD-ILD having 7(53.8%) out of 13 cases showed desaturation. More studies are required from developing countries to ascertain the spectrum of DPLDs in different geographic. Conclusion: On average, the 6MWT elicits a high but submaximal oxygen uptake in people with DPLD. Fibrosis is associated with maximum destauration in 6MWT. However the physiological load varies between individuals, with higher peak VO2 in those with more severe disease that may match or exceed that achieved on CPET. The 6MWT is not always a test of submaximal exercise capacity in people with DPLD.

Clinicoradiological Profile of Patients with Diffuse Parenchymal Lung Disease

The profile of diffuse parenchymal lung disease (DPLD) has mainly been reported from the developed countries; the prevalence and outcome of DPLD is not studied well across India. The aim of this study is to describe the clinical radiological spectrum of various DPLDs from a tertiary care centre of a developing country. Methods: An observational cross-sectional study was carried out in a single tertiary care center in northern India which included 50 consecutive subjects (age >12 years) who had diffuse parenchymal lung diseases. In this study, the clinical, radiological and histological data subjects was collected. Results: Out of the total 50 subjects, mean age was 49.04 years with females constituting 58% of the total. Most common symptoms were gradually progressing dyspnoea (100%) followed by dry cough (76%). The commonest finding on chest radiograph in our series was reticular pattern (78%).The commonest pattern on high-resolution computed tomography was traction bronchiectasis (56%), followed by honeycombing (50%), septal thickening (46%).The commonest cause of DPLD in study was Idiopathic Pulmonary Fibrosis (38) %, followed by connective tissue – interstitial lung disease (CTD-ILD)(26%) and NSIP(12%). Smoking was one of the major risk factor associated with IPF, and 52.6% of patients with IPF were smokers (P<0.001). Conclusion: IPF (38%) was the commonest DPLD seen followed by CTD- ILD and NSIP at a tertiary center in northern India similar to the spectrum reported from developed countries. More studies are required from developing countries to ascertain the spectrum of DPLDs in different geographic

Enfermedad pulmonar difusa: causa de hipertensión pulmonar persistente antes del año de vida / Diffuse lung disease: cause of persistent pulmonary hypertension before one year of age

En pediatría, la enfermedad vascular pulmonar es multifactorial y heterogénea. Si bien comparte algunas características con la hipertensión pulmonar en los adultos, hay diferencias en las comorbilidades y condiciones asociadas, la coexistencia de enfermedades genéticas o del desarrollo. Las enfermedades pulmonares intersticiales pueden ser causantes de esta entidad. Una de ellas es la displasia alvéolo-capilar con mal alineamiento de las venas pulmonares, una patología infrecuente pero con 100% de mortalidad, caracterizada por la falla en la formación del tejido pulmonar que da por resultado final la alteración en la difusión de gases. Se describe un caso clínico de una paciente de 5 meses de edad estudiada a partir de sospechar una cardiopatía congénita con hipoxemia persistente, a cuyo diagnóstico se llega por la biopsia pulmonar.

Pulmonary vascular disease in children is multifactorial and heterogeneous. While it shares some features with pulmonary hypertension in adults, there are differences in the associated comorbidities and conditions, the coexistence of genetic or developmental diseases. Interstitial lung diseases may be responsible for this entity. One is alveolar capillary dysplasia with misalignment of pulmonary veins, a rare pathology but with a mortality rate of 100%, characterized by a failure in the formation of lung tissue that eventually results in impaired gas diffusion. We present a 5-month-old patient studied due to suspected congenital heart disease with persistent hypoxemia; diagnosis was made through lung biopsy.

Diagnostic value and safety analysis of thoracoscopic lung biopsy in diffuse parenchymal lung disease / 中国内镜杂志

Objective To investigate the clinical value and safety of thoracoscopic lung biopsy in the diagnosis of diffuse parenchymal lung diseases. Methods 35 patients with diffuse parenchymal lung disease underwent thoraco-scopic lung biopsy were reviewed in order to obtain clinical and pathological data. Results 31 cases were diagnosed by pathology, and the total positive diagnostic rate was 88.6 % (31/35), 10 cases as usual interstitial pneumonial (UIP), 6 cases as Tuberculosis, 4 cases as alveolar cell carcinoma, 3 cases as nonspecific interstitial pneumonia (NSIP), 3 cases as crypto-genieorganizingpneumonia (COP), 2 cases as pulmonary alveolar proteinosis (PAP), 1 case as Wegener's granulomatosis (WG), 1 case as acute interstitial pneumonia (AIP), 1 case as lymphangioleiomyomatosis (LAM). 3 cases (8.6 %) were occurred with postoperative complications, 2 cases as pulmonary infection combined with respiratory failure, 1 case as idiopathic pulmonary fibrosis with acute exacerbation, and 2 cases (5.7 %) were complicated with death. Conclusions Thoracoscopic lung biopsy is a safe and effective method which helps final di-agnosis of diffuse parenchymal lung disease.

A rare case of ulcerative colitis with diffuse parenchymal lung disease, spontaneous pneumomediastinum and subcutaneous emphysema.

A 50-year-old male, a tobacco smoker, who was known to have ulcerative colitis presented with dry cough, chest pain, dysponea and frequent passage of blood and mucous mixed stools. Physical examination revealed clubbing, subcutaneous emphysema of upper chest and auscultatory findings of crunching sound over pre-cordial area and basal crepitations. Spirometry was suggestive of restrictive pattern. High resolution computed tomography (HRCT) of thorax revealed pneumomediastinum, subcutaneous emphysema, bilateral diffuse centrilobular nodules and ground-glass haziness with mosaic pattern along with posterior basal fibrotic changes. The present case documents the uncommon pulmonary involvement of spontaneous pneumomediastinum and subcutaneous emphysema diffuse parenchymal lung disease, in a patient with ulcerative colitis.

Terapia biológiaa en patología pulmonar: parte i generalidades y clasificaciónn parte ii: terapias específicas en asma, epoc, enfermedad pulmonar parinquematosa difusa, cáncer pulmonar / Terapiabiológicaa em patología pulmonar / Terapia biológica en patología pulmonar parte I: generalidades y clasificación parte II: terapias específicas en asma, EPOC, enfermedad pulmonar parinquematosa difusa, cáncer pulmonar

El avance de la investigación médica, en los campos de la biología molecular y la ingeniería genética, ha traído consigo el desarrollo de una serie de nuevos medicamentos dirigidos a bloquear diferentes vías de la respuesta inmune celular. La terapia biológica, nombre con el cual se reconoce a estos nuevos medicamentos, ofrece una nueva oportunidad terapéutica para el manejo de enfermedades crónicas progresivas. En las enfermedades pulmonares crónicas como el asma, la enfermedad pulmonar obstructiva crónica (EPOC), la enfermedad pulmonar parenquimatosa difusa (EPPD) y el cáncer de pulmón, el tratamiento con medicamentos biológicos ha aportado importantes avances para comprender con mayor claridad estas enfermedades y en algunos casos gracias a la eficacia de los mismos, mejorar la calidad de vida de los pacientes que las presentan. Debido al número cada vez mayor de medicamentos de terapia biológica y su aplicación terapéutica creciente en enfermedades inflamatorias crónicas y cáncer, creemos necesario revisar su estado actual en el manejo de la patología pulmonar crónica.

The advancement of medical research in molecular biology and genetic engineering has given rise to the development of new drugs aimed at blocking different pathways of cellular immune responses. Biological therapy is a new therapeutic option for progressive chronic disease management. In chronic lung diseases such as asthma, chronic obstructive pulmonary disease (COPD), diffuse parenchymal lung disease (DPLD) and lung cancer, treatment with biologics has made important advances in the understanding of these diseases, and in some cases, due to their effectiveness, has contributed to the improvement in life quality of patients who suffer them. Due to the increasing number of biological therapy drugs and their therapeutic application in chronic inflammatory diseases and cancer, it is relevant to review their current status in the management of chronic lung diseases.

O avanço da pesquisa médica, nos campos da biologia molecular e da engenharia genética, trouxe consigo o desenvolvimento de uma série de novos medicamentos dirigidos a bloquear diferentes vias da resposta imune celular. A terapia biológica, nome com o qual são conhecidos estes novos medicamentos, oferece uma nova oportunidade terapêutica para o tratamento de doenças cônicas progressivas. Nas doenças pulmonares crônicas como a asma, a doença pulmonar obstrutiva crônica (DPOC), a doença parenquimatosa difusa pulmonar (DPDP) e o câncer de pulmão, o tratamento com medicamentos biológicos tem contribuído com importantes avanços para compreender com maior claridade estas doenças e em alguns casos graças à eficácia dos mesmos, melhorar a qualidade de vida dos pacientes que as apresentam. Devido ao número cada vez maior de medicamentos de terapia biológica e sua aplicação terapêutica crescente em doenças inflamatórias crônicas e câncer, acreditamos que é necessário revisar seu estado atual no tratamento da patologia pulmonar Crônica.