British Thoracic Society study of cryptogenic fibrosing alveolitis: current presentation and initial management. Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society

Abstract

Abstractprognosis. Physicians generally considered CFA to be a clinical diagnosis and Background -Mortality due to cryptodid not initiate treatment in up to half of genic fibrosing alveolitis (CFA) is inpatients at presentation. creasing, particularly in the elderly. (Thorax 1997;52:38-44) Optimum management remains uncertain and previous studies of the disease have Keywords: cryptogenic fibrosing alveolitis, presentation, largely been from specialist centres. A treatment, British Thoracic Society.national study was carried out of the presentation and initial management of CFA in the UK.In England and Wales mortality due to cryptoMethods -All respiratory physicians in genic fibrosing alveolitis (CFA, also known as England, Scotland and Wales were invited idiopathic pulmonary fibrosis) doubled beto enter patients with newly diagnosed tween 1979 and 1988 1 and continues to in-CFA over a two year period. CFA was crease in the UK, Australia, and Canada. 2 The diagnosed on histological grounds or acincrease in mortality in the elderly is particularly cording to clinical criteria which included striking and the median age at diagnosis has the absence of a defined connective tissue been most recently reported as being in the disorder or pneumoconiosis. Participating seventh decade. 3 4 This compares with earlier physicians (n=150) completed a quesreports of the disease presenting in the fifth or tionnaire at patient entry and at all subsixth decade. [5][6][7] There are indications that the sequent follow up visits and death.disease is frequently diagnosed on clinical Results -A total of 588 patients (373 men, grounds 3 despite previous recommendations 63%) were studied of whom 441 (75%) were that histological data should be obtained. to initiate treatment was made pre-ticipate in a study designed by the Research dominantly on symptomatic grounds. Two

The effect of lung biopsy on lung function in diffuse lung disease

“…In one series, only 12% of patients with suspected CFA underwent OLB. Age and poor lung function were the most important factors that led to the decision to avoid this investigation [10].…”

“…Longer-term effects such as those on lung function have not been studied. It has been suggested that lung biopsy may cause a deterioration in lung function and this may partly explain the reluctance of many physicians in the UK to include lung biopsy in the evaluative process [10]. The goals of the present study were to examine the effects of lung biopsy on lung function and to compare the consequences of OLB and VATS.…”

Idiopathic pulmonary fibrosis: current understanding of the pathogenesis and the status of treatment

“…The most common clinical presentation of IPF is that of insidious progressive shortness of breath, or dyspnea, being present for at least 3 to 4 months. 3,4,51 Cough may be present with or without sputum. 3,4,51 On physical examination 25%-50% of patients with IPF have evidence of clubbing.…”

“…3,4,51 On physical examination 25%-50% of patients with IPF have evidence of clubbing. [3][4][5]51 The presence of clubbing is helpful in suggesting a diagnosis of 4 ‡Clinical disorder that corresponds to the histological pattern indicated on the left. 4 §Provisional nomenclature subject to further classification.…”

Adult-onset pulmonary fibrosis caused by mutations in telomerase

“…The inheritance pattern is most consistent with autosomal dominant with incomplete penetrance. The clinical presentation of familial IPF is indistinguishable from sporadic IPF except that the age of onset tends to be earlier (55 years vs. 67 years) (4,5). Here we report the use of a genetic linkage approach to map the culprit gene in two large families to chromosome 5.…”