Crackles

Abstract: In 50 of 94 patients with bronchiolitis obliterans we found no apparent cause or associated disease, and the bronchiolitis obliterans occurred with patchy organizing pneumonia. Histologic characteristics included polypoid masses of granulation tissue in lumens of small airways, alveolar ducts, and some alveoli. The fibrosis was uniform in age, suggesting that all repair had begun at the same time. The distribution was patchy, with preservation of background architecture. Clinically, there was cough or flu-like illness for 4 to 10 weeks, and crackles were heard in the lungs of 68 per cent of the patients. Radiographs showed an unusual pattern of patchy densities with a "ground glass" appearance in 81 per cent. Physiologically, there was restriction in 72 per cent of the patients, and 86 per cent had impaired diffusing capacity. Obstruction was limited to smokers. The mean follow-up period was four years. With corticosteroids, there was complete clinical and physiologic recovery in 65 per cent of the subjects; two died from progressive disease. This disorder differs from bronchiolitis obliterans with irreversible obstruction. It was confused most often with idiopathic pulmonary fibrosis. In view of the benign course and therapeutic response, a histologic distinction is important.

Abstract: CLINICAL SCENARIO You are a medical consultant asked by a surgical colleague to see a 78-year-old woman, now 10 days after abdominal surgery, who has become increasingly short of breath over the last 24 hours. She has also been experiencing what she describes as chest discomfort, which is sometimes made worse by taking a deep breath (but sometimes not). Abnormal findings on physical examination are restricted to residual tenderness in the abdomen and scattered crackles at both lung bases. Chest roentgenogram reveals a small right pleural effusion, but this is the first roentgenogram since the operation. Arterial blood gases show a Po 2 of 70 mm Hg, with a saturation of 92%. The electrocardiogram shows only nonspecific changes. You suspect that the patient, despite receiving 5000 U of heparin twice a day,

Abstract: OBJECTIVE—To describe the survival of a population based cohort of patients with incident (new) heart failure and the clinical features associated with mortality. DESIGN—A population based observational study. SETTING—Population of 151 000 served by 82 general practitioners in west London. PATIENTS—New cases of heart failure were identified by daily surveillance of acute hospital admissions to the local district general hospital, and by general practitioner referral of all suspected new cases of heart failure to a rapid access clinic. INTERVENTIONS—All patients with suspected heart failure underwent clinical assessment, and chest radiography, ECG, and echocardiogram were performed. A panel of three cardiologists reviewed all the data and determined whether the definition of heart failure had been met. Patients were subsequently managed by the general practitioner in consultation with the local cardiologist or admitting physician. MAIN OUTCOME MEASURES—Death, overall and from cardiovascular causes. RESULTS—There were 90 deaths (83 cardiovascular deaths) in the cohort of 220 patients with incident heart failure over a median follow up of 16 months. Survival was 81% at one month, 75% at three months, 70% at six months, 62% at 12 months, and 57% at 18 months. Lower systolic blood pressure, higher serum creatinine concentration, and greater extent of crackles on auscultation of the lungs were independently predictive of cardiovascular mortality (all p < 0.001). CONCLUSIONS—In patients with new heart failure, mortality is high in the first few weeks after diagnosis. Simple clinical features can identify a group of patients at especially high risk of death. Keywords: heart failure; prognosis; survival; epidemiology

Abstract: Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000) than in women (13.2/100,000). The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock). IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP). The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis), forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational) exposures. IPF is typically progressive and leads to significant disability. The median survival is 2 to 5 years from the time of diagnosis. Medical therapy is ineffective in the treatment of IPF. New molecular therapeutic targets have been identified and several clinical trials are investigating the efficacy of novel medication. Meanwhile, pulmonary transplantation remains a viable option for patients with IPF. It is expected that, during the next decade, considerable progress will be made toward the understanding and treatment of this devastating illness.