Complete Atrioventricular Block and Cardiopulmonary Involvement in Rapidly Progressive Systemic Sclerosis

Abstract The heart and lung are target organs in systemic sclerosis (SSc) and similar symptoms (dyspnea and cough) may make the differential diagnosis between the two lesions difficult. In addition, complete atrioventricular block (CAVB) is a rare complication of this disease. This case report is about a patient with SSc and pulmonary fibrosis who was admitted to the emergency room with CAVB, heart failure (HF) and progressive worsening of the underlying disease.

Artritis reumatoidea y síndrome combinado de fibrosis pulmonar y enfisema / Rheumatoid arthritis and combined pulmonary fibrosis and emphysema

La combinación de fibrosis pulmonar y enfisema es un síndrome descripto en los últimos años que tiene características propias y no es la casual asociación de dos entidades. El componente de fibrosis más común corresponde a la fibrosis pulmonar idiopática. Sin embargo, otras enfermedades intersticiales pueden formar parte de este síndrome, entre ellas las asociadas a enfermedades del tejido conectivo. Se presenta un caso de este síndrome asociado a artritis reumatoidea con la particularidad que la misma se hizo evidente varios años después del síndrome combinado fibrosis pulmonar y enfisema, hecho muy poco comunicado en la literatura.

The combination of pulmonary fibrosis and emphysema is a syndrome described in the last years, which has its own characteristics and it is not only the casual association between the two entities. The idiopathic pulmonary fibrosis is the most common type of pulmonary fibrosis. However other interstitial lung diseases could be part of this syndrome. Among them is the connective tissue disease-associated interstitial lung disease. We report a case of this syndrome associated with rheumatoid arthritis. It has the peculiarity that the connective disease became overt several years after the presentation of combined pulmonary fibrosis and emphysema syndrome, which is infrequently reported in the literature.

Aproximación diagnóstica a las enfermedades pulmonares difusas / Diagnostic approach to diffuse pulmonary diseases

Las enfermedades pulmonares difusas representan un grupo de enfermedades que comparten un mismo criterio radiológico, existiendo más de 200 entidades que se presentan como tal. La clínica es fundamental para aproximar el diagnóstico etiológico que muchas veces resulta complejo. Tos y disnea progresiva son los síntomas clínicos característicos de estas enfermedades y se acompañan de la radiografía de tórax con opacidades difusas como método radiológico inicial. El estudio en general es multi- disciplinario incluyendo patrones radiológicos de la tomografía axial de tórax, estudio de función pulmonar, lavado bronquioal- veolar y biopsia pulmonar en algunos casos.

The diffuse lung diseases are a group of conditions that share common radiological criteria. There are over 200 causes. The clinic skill is essential to approximate the etiologic diagnosis, often complicated. Cough and progressive dyspnea are the clinical features of these diseases and are accompanied by chest radiography with diffuse opacities as the initial radiological method. The study is generally multidisciplinary and including radiological patterns in computer tomography of the chest, lung function study, bronchoalveolar lavage and lung biopsy in some cases.

Combined pulmonary fibrosis and emphysema with pulmonary arterial hypertension: A Rare Case Report.

Combined pulmonary fi brosis and emphysema (CPFE) is a recently recognized radiologically defi ned syndrome characterized by simultaneous coexistence of both upper lobe emphysema and lower lobe pulmonary fi brosis. We present a case of CPFE in a 57-year-old male smoker who presented with dry cough, progressive breathlessness, and swelling of feet. His chest X-ray revealed bilateral lower Zone reticulonodular opacities with hyperlucent upper Zones. Pulmonary function tests showed wellpreserved lung volumes and reduced diff using capacity of the lung for carbon monoxide. High-resolution computed tomogram showed bilateral lower lobe subpleural honeycombing along with fi brosis and traction bronchiectasis with bilateral upper lobe emphysema predominantly paraseptal type. His 2D echo was suggestive of moderate pulmonary arterial hypertension.

Pulmonary fibrosis and emphysema.

A 58-year-old male presented with symptoms of progressive dyspnoea on exertion and predominantly dry cough of six months duration. He was a cigarette smoker with a smoking index of 10 pack years. He had no history of fever, exposure to dusts or drug therapy. There was no history of similar illness in the family members or any symptoms suggestive of connective tissue disease (CTD). On physical examination, clubbing was observed and there were bibasilar fine endinspiratory crackles on auscultation. Oxygen saturation by pulse oximetry showed significant exercise desaturation from 94% to 77%.

Fibrose pulmonar idiopática: uma atualização / Idiopathic pulmonary fibrosis: an update

A fibrose pulmonar idiopática (FPI) é uma das doenças pulmonares intersticiais mais comuns. Tem caráter crônico, progressivo, restringe-se ao pulmão e é de causa desconhecida, acometendo preferencialmente adultos a partir da meia idade. A FPI é uma entidade nosológica distinta; histologicamente, se manifesta no padrão de pneumonia intersticial usual (PIU). Na presença de achados típicos do padrão PIU tomográfico, a TCAR de tórax tem elevada acurácia em predizer o achado histológico de PIU e, nesses casos, o achado tomográfico típico é suficiente para o diagnóstico de FPI dentro de um contexto clínico adequado. Em relação ao tratamento, a necessidade de diferenciação da FPI dentre as outras doenças intersticiais pulmonares vem crescendo devido ao fato de recentes evidências mostrarem um aumento de mortalidade e hospitalizações naqueles pacientes com FPI tratados com azatioprina e prednisona. Além disso, novas drogas antifibróticas de uso restrito na FPI estão surgindo.

Artropatía de Jaccoud y fibrosis pulmonar en síndrome CREST / Jaccoud’s arthropathy and pulmonary fibrosis in CREST syndrome

Se describe el caso de una paciente de 48 años de edad con diagnóstico de síndrome CREST incompleto (variante de esclerosis sistémica limitada), en quien se documenta la presencia de artropatía de Jaccoud de las manos y compromiso pulmonar intersticial por fibrosis pulmonar tipo neumonía intersticial usual, con positividad para factor reumatoide y anticuerpos contra el péptido citrulinado.Palabras clave: síndrome CREST, artropatía, enfermedad pulmonar intersticial, fibrosis pulmonar.

We report a case of a 48 years old patient with diagnosis of incomplete CREST syndrome (variant limited systemic sclerosis) in who we documented the presence of Jaccoud’s arthropathy of the hands and pulmonary involvement by pulmonary fibrosis type usual interstitial pneumonia, with positivity for rheumatoid factor and anti-cyclic citrullinated peptide antibody.Key words: CREST syndrome, joint disease, interstitial lung disease, pulmonary fibrosis.

Factores predictores de la caída de la saturación de oxígeno durante la caminata de 6 minutos en la fibrosis pulmonar idiopática / Predictors factors of the fall of oxygen saturation during the six-minute walk test in idiopathic pulmonary fibrosis

La prueba de caminata de seis minutos (PC6m) es potencialmente útil en la fibrosis pulmonar idiopática (FPI), ya que proporciona información para el pronóstico. El objetivo de nuestro estudio fue determinar si las pruebas funcionales pulmonares en reposo pueden predecir la disminución de la saturación de oxígeno (SaO2) durante una prueba PC6m y si la distancia caminada se correlaciona con las pruebas funcionales pulmonares en reposo. Se incluyeron 53 pacientes con diagnóstico de FPI de acuerdo a la definición de la ATS/ERS a los que se les realizó espirometría, medición de volúmenes pulmonares, DLCO y PC6m. Los metros caminados en la PC6m no se correlacionaron con la edad (r = -0.154; p = 0.272), ni con la DLCO en % del valor predicho (r = 0.261, p =0.065), pero en cambio se correlacionaron con la SaO2 basal (r = 0.359; p = 0.007). Los pacientes que mostraron caída de la SaO2 no fueron diferentes de los que no mostraron desaturación en términos de presencia de disnea en el momento del diagnóstico ( 78 vs 66 % ; p=0.124), presencia de una TAC catalogada como neumonía intersticial usual definitiva (72% vs 80%; p = 0.52) o proporción de pacientes con CVF normal como % del valor predicho (59% vs 77%; p = 0.29) pero tuvieron diferencias estadísticamente significativas en la CVF (61.80 ±17.50 vs. 82.50 ±19.02; p = 0.003) y en la saturación basal (94.03 ±1.98 vs. 95.87 ± 2.17; p= 0.004). Como conclusión la caída de la SaO2 durante la PC6m no puede predecirse por los valores de la DLCO ni por las anomalías encontradas en la TAC de tórax pero se correlaciona con la disminución de la CVF. Este estudio enfatiza la importancia de evaluar la caída de la SaO2 en la FPI independientemente de la disnea o la DLCO.

The six-minute walk-test (6mWT) is potentially useful to define prognosis in patients with idiopathic pulmonary fibrosis (IPF). The objective of this study was to determine if resting pulmonary function tests (PFT) can predict the presence of decrease of oxygen saturation (SaO2) during a 6mWT and if the walked distance correlates with the resting PFT. Fifty-three patients with IPF were included according to the ATS/ERS consensus definition. Pulmonary function tests (including spirometry, lung volumes and diffusing lung capacity of carbon monoxide - DLCO) and a 6mWT were performed in all patients. The walked distance during a 6mWT did not correlate with age (r = -0.154; p = 0.272) or DLCO as % of predicted value (r = 0.261, p = 0.065), but it correlated with baseline SaO2 (r = 0.359; p = 0.007). No significant difference was found in patients who showed fall of oxygen saturation in comparison with those who did not in the proportion of the following variables: dyspnea at the moment of diagnosis (78 vs. 66%; p = 0.124), high resolution computed tomography (HRCT) showing definitive usual interstitial pneumonia (72% vs. 80%; p = 0.52) and normal FVC as % of predicted value (59 % vs. 77 %; p = 0.29). In contrast, there was a significant difference in FVC (61.80 ± 17.50 vs. 82.50 ± 19.02, p = 0.003) and in basal saturation (94.03 ± 1.98 vs. 95.87 ± 2.17, p = 0.004) between these two groups. The fall of SaO2 during the 6mWT cannot be predicted by clinical variables or HRCT findings but it does relate to the fall of FVC. This study shows the importance of performing the 6mWT independently of the presence of dyspnea or spirometry results.

Idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is being more frequently diagnosed in India, due to its increased awareness, better availability of computed tomography (CT) and fiberoptic bronchoscopy. IPF has the histological appearance of usual interstitial pneumonia (UIP) on surgical lung biopsy. Recent research has given a new insight into the etiology of the disease. Clinical criteria have been specified for presumptive diagnosis of IPF and distinguishing IPF from other conditions. The conventional therapy has been steroids and immunosuppressive agents. But only a minority of patients respond to such a therapy. Immunomodulators (interferon Y1b), antioxidants (Acetyl cysteine) and antifibrotic agents (like pirfenidone) are being studied as novel therapies in this, otherwise, fatal condition. Lung transplantation is the only hope for those patients who show progressive deterioration on medical treatment. Living-donor lobar lung transplantation has been developed as a procedure for patients considered too ill to await cadaveric lung transplantation.

Idiopathic pulmonary fibrosis: a decade of progress

Embora diagnósticos de fibrose pulmonar idiopática continuem sendo devastadores, avanços recentes têm melhorado nossa compreensão a respeito de muitas das facetas desta doença. Estas descobertas, juntamente com o aumento da disponibilidade geral de ensaios terapêuticos, encerram a promessa de um futuro mais promissor para pacientes com fibrose pulmonar idiopática. Por exemplo, nós temos agora uma compreensão mais abrangente a respeito dos critérios diagnósticos e da história natural da doença. Vários estudos têm mostrado que a mensuração simples da fisiologia pulmonar ou troca gasosa pode ser usada para prever a sobrevida do paciente. Através da identificação de várias vias moleculares que têm papéis importantes na patogênese da fibrose pulmonar idiopática, os pesquisadores têm produzido uma lista crescente de possíveis novos alvos terapêuticos para a doença. Vários ensaios terapêuticos prospectivos e controlados têm sido realizados. Outros estão em andamento ou ainda estão em fase de planejamento. Estes esforços têm avançado nosso conhecimento atual sobre fibrose pulmonar idiopática e levantado novas questões importantes, assim como têm gerado o interesse e o impulso necessários para avançar terreno na luta contra esta doença desafiadora. Este artigo oferece ao leitor um panorama dos avanços recentes nas pesquisas sobre fibrose pulmonar idiopática, tendo como foco a história natural, patogênese e tratamento.

Although idiopathic pulmonary fibrosis remains a devastating diagnosis, recent advances have improved our understanding of many facets of this disease. These breakthroughs, combined with the increased general availability of therapeutic trials, hold the promise of a brighter future for idiopathic pulmonary fibrosis patients. For example, we now have a more comprehensive understanding of the diagnostic criteria and natural history of the disease. Several studies have shown that simple measurement of pulmonary physiology or gas exchange can be used to predict patient survival. By identifying several molecular pathways that play significant roles in the pathogenesis of idiopathic pulmonary fibrosis, investigators have produced a growing list of novel potential therapeutic targets for the disease. Several prospective, controlled therapeutic trials have been conducted. Others are ongoing or are still in the planning stages. These efforts have advanced our current knowledge of idiopathic pulmonary fibrosis and have raised new important questions, as well as having generated the interest and momentum needed to gain additional ground in the fight against this challenging disease. This article offers the reader a view of the recent advances in idiopathic pulmonary fibrosis research, with a focus on natural history, pathogenesis and treatment.

Manifestações extra-esofágicas da doença do refluxo gastroesofágico / Extraesophageal manifestations of gastroesophageal reflux disease

A doença do refluxo gastroesofágico freqüentemente se apresenta com pirose e regurgitação, os chamados sintomas típicos. Porém, um subgrupo de pacientes apresenta um conjunto de sinais e sintomas que não estão relacionados diretamente ao dano esofágico. A esse conjunto dá-se o nome de manifestações extra-esofágicas da doença do refluxo gastroesofágico. Compreendem, principalmente, broncoespasmo, tosse crônica e alterações inflamatórias na laringe (chamados manifestações atípicas). Apesar de essas manifestações formarem um grupo heterogêneo, algumas considerações gerais englobam todos os subgrupos: embora a associação entre a doença do refluxo gastroesofágico e as manifestações extra-esofágicas esteja bem estabelecida, uma relação entre causa e efeito definitiva ainda não está elucidada; em relação à patogênese das manifestações extra-esofágicas, os principais mecanismos propostos são a injúria direta do tecido extra-esofágico pelo conteúdo ácido gástrico refluído e o reflexo esôfago-brônquico mediado pelo nervo vago; a doença do refluxo gastroesofágico pode não ser incluída no diagnóstico diferencial do grupo de pacientes que apresenta somente os sintomas atípicos. Este artigo revisa as manifestações extra-esofágicas da doença do refluxo gastroesofágico encontradas na literatura, discutindo a epidemiologia, patogênese, diagnóstico e tratamento, com foco nas apresentações mais estudadas e estabelecidas.

Fibrose pulmonar idiopática: comparação de dados clínicos e funcionais em pacientes com e sem faveolamento / Idiopathic pulmonary fibrosis: comparison of clinical and functional findings in patients with anda without honeycombing

Introdução: faveolamento é um importante fator preditivo de mortalidade na fibrose pulmonar idiopática (FPI). O objetivo deste estudo foi comparar os achados clínicos e funcionais em pacientes com e sem faveolamento. Metodologia: foi realizado um estudo de corte transversal, em que foram avaliados 41 pacientes não tabagistas, portadores de FPI. Todos os pacientes responderam um questionário padronizado (incluindo avaliação de tempo de sintomas e dispnéia) e realizaram radiografia de tórax, tomografia computadorizada de alta resolução (TCAR), espirometria, oscilações forçadas, diluição com hélio e difusão com monóxido de carbono (DCO). Para uma análise mais específica das radiografias e dos exames de TCAR, os pacientes foram divididos em dois grupos: grupo A (presença de faveolamento) e grupo B (ausência de faveolamento). Resultados: dos 41 pacientes estudados, 26 eram mulheres e 15 eram homens, com média de idade de 70,8 anos. A radiografia convencional do tórax e a TCAR demonstraram o faveolamento em 26 e 34 casos, respectivamente. Houve razoável concordância entre os dois métodos na capacidade de mostrar esse tipo de anormalidade (φ=0,59; p<0,001). Foram observadas diferenças significativas na complacência dinâmica do sistema respiratório (Crs,dyn), DCO e capacidade pulmonartotal (CPT) entre os pacientes com e sem faveolamento, tanto na radiografia convencional do tórax quanto na TCAR. Para as variáveis clínicas, apenas a dispnéia associou-se ao grupo com faveolamento na radiografia de tórax. Conclusão: em portadores de FPI, os parâmetros funcionais refletem a intensidade do processo morfológico subjacente mais acuradamente queas manifestações clínicas da doença.

Esclerose sistêmica progressiva: aspectos na tomografia computadorizada de alta resolução / Progressive systemic sclerosis: high-resolution computed tomography findings

OBJETIVO: Descrever os achados de tomografia computadorizada de alta resolução de pacientes com esclerose sistêmica pulmonar, independentemente dos sintomas respiratórios. MATERIAIS E MÉTODOS: Foram revisados 73 exames de tomografia computadorizada de alta resolução de 44 pacientes com diagnóstico de esclerodermia estabelecido através de critérios clínicos e laboratoriais. Os exames foram revisados por dois radiologistas, que estabeleceram os achados por consenso. RESULTADOS: Em 91,8 por cento (n = 67) dos exames observaram-se alterações. Os principais achados foram lesões de padrão reticular (90,4 por cento), opacidades em vidro-fosco (63 por cento), bronquiectasias e bronquiolectasias de tração (56,2 por cento), dilatação esofagiana (46,6 por cento), faveolamento (28,8 por cento) e sinais de hipertensão pulmonar (15,6 por cento). Na maioria dos casos as lesões eram bilaterais (89 por cento) e simétricas (58,5 por cento). Quanto à localização, houve predomínio de lesões basais (91,2 por cento) e periféricas (92,2 por cento). CONCLUSÃO: A esclerose sistêmica progressiva acarreta fibrose pulmonar na maioria dos pacientes, caracterizada principalmente por lesão reticular basal e periférica.

OBJECTIVE: To describe the high-resolution computed tomography findings in the lung of patients with systemic sclerosis, independently of the respiratory symptoms. MATERIALS AND METHODS: Seventy-three high-resolution computed tomography scans of 44 patients with clinical diagnosis of systemic sclerosis were reviewed and defined by the consensus of two radiologists. RESULTS: Abnormalities were seen in 91.8% (n = 67) of the scans. The most frequent findings were reticular pattern (90.4%), ground-glass opacities (63%), traction bronchiectasis and bronchiolectasis (56.2%), esophageal dilatation (46.6%), honeycombing pattern (28.8%) and signs of pulmonary hypertension (15.6%). In most cases the lesions were bilateral (89%) and symmetrical (58.5%). The lesions were predominantly located in the basal (91.2%) and peripheral (92.2%) regions. CONCLUSION: In the majority of the patients, progressive systemic sclerosis can cause pulmonary fibrosis mainly characterized by reticular pattern with basal and peripheral distribution on high-resolution computed tomography.

Insuficiencia respiratoria progresiva secundaria a fibrosis pulmonar en una escolar: Caso clínico / Pulmonary fibrosis in childhood: Report of one case

Interstitial lung diseases are uncommon in children, and can be idiopathic or secondary to known causes, sharing common pathological findings. We report a girl with progressive respiratory insufficiency secondary to interstitial pneumonia and pulmonary fibrosis, with risk factors such as bronchopulmonary dysplasia and respiratory infections (respiratory syncytial virus and suspected Mycoplasma pneumoniae), that may have had an additive effect. Nasal bi-level Positive Airway Pressure was used in the last period of her disease. She died due to global respiratory failure at the age of 14 years.

A comparative study on idiopathic pulmonary fibrosis and secondary diffuse parenchymal lung disease.

BACKGROUND AND AIMS: Clinical characteristics of patients diagnosed to have Diffuse parenchymal lung disease (DPLD) were evaluated in this study. DESIGN AND SETTING: Retrospective evaluation, a tertiary care center in South India. MATERIAL AND METHOD: Subjects diagnosed to have DPLD over a five-year period were included in this study. Data pertained to clinical characteristics and lab parameters were obtained. STATISTICAL CONSIDERATIONS: t- test for Mean values and chi-square test for comparing proportions were used. RESULTS: There were 73 eligible patients included for evaluation. Secondary cause for DPLD was diagnosed in 40 (55%) and idiopathic pulmonary fibrosis (IPF) was diagnosed in 33 (45%). The mean age was 45+/-11 and 53+/-10 years, of these 5 (12%) and 17 (52%) were male subjects in the secondary DPLD and IPF group respectively. The mean age, dyspnoea, cough, clubbing and crepitations were noted to be higher in patients with IPF as compared to patients with secondary DPLD. Fifty patients were followed up for a mean of 13 months (28 secondary DPLD and 18 IPF). Follow up data was available in 46 patients. Of these subjects prednisone alone was initiated in 24 subjects and combination with azathioprine in 22. Subjective improvement in symptoms was noted in 29/46 (63%), 19 with secondary DPLD and 10 with IPF. CONCLUSION: symptoms and signs were noted more frequently with IPF, subjective improvement to treatment was noted in 63% and the best response was noted among patients diagnosed to have sarcoidosis. A prospective trial is needed to study the long term prognosis and therapeutic response among Indian patients.

Spectrum and diagnosis of idiopathic pulmonary fibrosis.

OBJECTIVE: To study the clinical profile of patients with idiopathic pulmonary fibrosis (IPF) and methods used for diagnosis. METHODS: Prospective analysis of symptoms, signs, radiology and lung biopsy of patients freshly diagnosed to have IPF over a 16-month period. RESULTS: During the study period, 76 patients (35 men) with a mean age of 50.6 +/- 11.9 years were diagnosed to have IPE Breathlessness (98.6%) and dry cough (92.1%) were the most common presenting symptoms. Transbronchial lung biopsy (TBLB) was performed in 38 (50%) patients. Histopathological examination revealed features consistent with IPF in 35 (92.1%) patients; two of the remaining three patients underwent open lung biopsy. Other patients were diagnosed based on clinical features and high resolution chest tomography (HRCT) findings. HRCT was performed in 69 (90.8%) patients; all had features suggestive of diffuse interstitial fibrosis. CONCLUSION: IPF is diagnosed more commonly now than in the past. Indian patients may be developing the disease a decade earlier than their counterparts in the West. TBLB and HRCT are useful in establishing diagnosis. IPF should be considered a distinct clinical entity rather than a diagnosis of exclusion.

Drug-induced pulmonary fibrosis

Pulmonary fibrosis is characterized by the accumulation of excessive connective tissue in the lungs. Its causes include chronic administration of some drugs for example bleomycin, cyclophosphamide, amiodarone, procainamide, penicillamine, gold and nitrofurantoin; exposure to certain environmental factors such as gases, asbestos and silica and bacterial or fungal infections. Some systemic diseases also predispose to the disease for example rheumatoid arthritis and systemic lupus erythematosus. The disease is associated with release of oxygen radicals and some mediators such as tumor necrosis factor-alpha TNF-alpha, transforming growth factor-beta TGF-beta, PDGF, IGF-I, ET-I and interleukins 1, 4, 8 and 13. The symptoms of the disease include dyspnea, non-productive cough, fever and damage to the lung cells. It is diagnosed with the aid of chest radiography, high resolution computed tomographic scanning and the result of pulmonary function tests. Drug-induced pulmonary fibrosis may involve release of free oxygen radicals and various cytokines for example IL-Ibeta and TNF-alpha via activation of nuclear transcription factor NF-beta as in the case of bleomycin and mitomycin or via release of TGF-beta as in case of tamoxifen or via inhibition of macrophages' and lymphocytes' phospholipases as in the case of amiodarone with the resultant accumulation of phospholipids and reduction of the immune system

Lymphocytic interstitial lung disorder: an isolated entity.

A 40 years female patient presented with recurrent haempoptysis since last five years and taking antituberculous as well as antidiabetic treatment. She was further investigated and found having lymphocytic interstitial lung disease without any other autoimmune disorder. She was treated by surgery with good response.