Thorax: first published as 10.1136/thx.26.3.300 on 1 May 1971. Downloaded from Thorax (1971), 26, 300. Diffuse pulmonary fibrosis and hiatus hernia J. E. G. PEARSON and R. S. E. WILSON The United Bristol Hospitals and Frenchay Hospital Six patients presenting with initially obscure pulmonary fibrosis are described. Reasons are given for attributing the condition to chronic aspiration pneumonitis from gastro-oesophageal reflux in association with hiatus hernia, supported by lung histology in three cases. Study of a further series of 143 cases of hiatus hernia suggests a 4% incidence of this insidious complication in addition to any more acute bronchopulmonary episodes. This possibility should be remembered in the differential diagnosis of any patient with pulmonary fibrosis of doubtful origin, as the advanced condition bears a poor prognosis. Although Plummer and Vinson (1921) emphasized aetiological factor (Scadding, 1956, 1960; Herbert, that patients with achalasia of the cardia were Nahmias, Gaensler, and MacMahon, 1962; liable to develop pulmonary complications, the Livingstone, Lewis, Reid and Jefferson, 1964; relationship between bronchopulmonary disease Reid, Cuthbert, and Craik, 1965; Stack, Grant, and oesophageal dysfunction was not widely Irvine, and Moffat, 1965). Castleman and Kibbee recognized until Vinson (1927) explained cause (1958) mentioned the presence of a hiatus hernia and effect in a case of achalasia complicated by in a patient dying of idiopathic pulmonary fibrosis a lung abscess. Aspiration of gastric and oeso- but drew no conclusion from this finding. phageal contents also occurs with pharyngeal It is not the intention here to mention the diverticulum, carcinoma of the oesophagus or acute or subacute cases of pneumonitis in this http://thorax.bmj.com/ cardia, inflammatory stricture, anaesthesia, and in condition but to emphasize how the chronic and patients with neurological disorders. Belcher (1949) insidious lesions may create diagnostic difficulty, reviewed 48 patients with dysphagia pneumonitis and to show that they seem to occur with gastro- of whom 10, all with achalasia, had pulmonary oesophageal reflux from hiatus hernia. By defini- fibrosis alone. Anderson, Holman, and Olsen tion the patients in this group are not known to (1953) reviewed 601 cases of achalasia, of whom have had any of the acute complications such as only three had pulmonary fibrosis as the sole lung abscess or infective 'pneumonitis', although lesion. Belsey (1960) reviewed 1,308 patients with these conditions may well lead to chronic disease oesophageal disease and found pulmonary com- such as bronchiectasis, which for the same reason on September 30, 2021 by guest. Protected copyright. plications in a considerable but unspecified num- is also not considered. ber, the commonest being aspiration pneumonitis characterized by haemoptysis, purulent sputum, CASE REPORTS pleuritic pain, and dyspnoea. Some residual fibrosis or bronchiectasis secondary frequently CASE 1 A 65-year-old housewife sought medical advice followed. Other complications included lung in 1960 with heartburn, flatulence, tightness in the abscess, haemoptysis, and collapse. Belsey also chest in the morning, slight dyspnoea, and a dry cough. commented that progressive pulmonary fibrosis, In 1941 the patient had been investigated for dyspepsia, not associated with an acute episode, was observed which in view of a negative barium meal and laparo- 'in a few cases'. However, the number of cases tomy was labelled 'functional dyspepsia'. There was and the associated oesophageal lesion was not no history of chest disease, arthritis, exposure to occu- stated. Bronchitis, peribronchial infection, pneu- pational dusts or fumes, or allergy, and she was a monitis, fibrosis of the lower lobes, and aspiration non-smoker. are of hiatus On examination slight kyphoscoliosis and scattered pneumonia recognized complications crepitations on the right were the only abnormal find- hernia (Naish and Read, 1965; Atkinson, 1967). ings. Her chest radiograph (Fig. 1) suggested scattered In spite of the association of hiatus hernia and fibrosis of the right lung and the left lower lobe. A lung disease, reviews of pulmonary fibrosis of barium meal revealed a hiatus hernia, and oeso- unknown cause do not consider it as a possible phagoscopy showed gastro-oesophageal reflux. 300 Thorax: first published as 10.1136/thx.26.3.300 on 1 May 1971. Downloaded from Dijfuse pulmonary fibrosis and hiatus hernia 301 w http://thorax.bmj.com/ FIG. 1. Chest radiograph of a 65-year-old woman showing scattered lesions in right lung anid left lower lobe. In March 1961 a hiatus hernia repair was per- crackling sounds over the lower half of both lung formed. This was followed by considerable sympto- fields. A chest radiograph was suggestive of fibrosis matic improvement, but by 1967 she was again com- of the right lower lobe, with scoliosis obscuring the on September 30, 2021 by guest. Protected copyright. plaining of dyspepsia and dyspnoea, though by now left side. The electrocardiogram was normal, and a she was aged 72. Latex fixation test was negative. CASE 2 A 62-year-old wife of a medical practitioner CASE 3 An intelligent 58-year-old businessman was presented with a 10-year history of gastro-oesophageal seen as an outpatient in 1959, with a three-year his- reflux in 1962. At that time she was noticed to have tory of a dry irritating nocturnal cough, relieved by persistent basal orepitations. A barium meal showed a lying on his left side. He confirmed that the cough hiatus hernia, which was repaired in April 1962. In related to meals and was associated with a congested 1969 she complained of an aching pain in the left side feeling in the chest, helped by magnesium trisilicate. of the chest, worse on sitting after exercise. The pain Because of this a barium meal was performed and was neither pleuritic nor anginal in type and was showed a hiatus hernia. Oesophagoscopy revealed thought to be a musculoskeletal disorder due to severe gastro-oesophageal reflux. He was a non-smoker, scoliosis and her thoracotomy scar. The patient had and gave no history of acute chest disease, arthritis, noticed increasing dyspnoea and a dry cough over the allergy or exposure to dusts or fumes. previous five years, and had suffered from occasional On examination finger clubbing was present, chest attacks of nocturnal laryngospasm which started 10 expansion was moderate, and there were scattered years prior to hiatus hernia repair, was absent for two bilateral crepitations over the middle and lower por- years after operation, and had since recurred. She gave tions of the lung. A chest radiograph (Fig. 2) showed no history of other chest disease, arthritis, exposure to diffuse mottling in both lung fields, maximal at the dusts or fumes, or allergy, and was a non-smoker. bases. An electrocardiogram was normal, and he was Examination showed gross scoliosis, with medium not anaemic. Thorax: first published as 10.1136/thx.26.3.300 on 1 May 1971. Downloaded from 302 J. E. G. Pearson and R. S. E. Wilson http://thorax.bmj.com/ FIG. 2. Chest radiograph showing diffuse mottling in a 58-year-old man. The hiatus hernia was repaired, and a biopsy was were negative; an electrocardiogram was normal, and taken from the extensively scarred lungs. Histology his barium meal showed a hiatus hernia but no duo- showed replacement of the lung tissue by fibrous denal ulceration. tissue containing a few lymphocytes. There was The patient's condition deteriorated rapidly and he alveolar epithelialization with some alveoli con- died in respiratory failure. At necropsy, the lower end taining clusters of macrophages. The pathologist of the oesophagus was dilated, but there was little on September 30, 2021 by guest. Protected copyright. reported 'typical chronic pneumonitis'. The patient's oesophagitis. IJhe lungs were emphysematous and condition slowly deteriorated, and he died of respira- showed extensive scarring. Histology confirmed wide- tory failure and cor pulmonale within two years. No spread irregular areas of interstitial fibrosis, with necropsy was obtained. areas where alveolar spaces were filled with fine fib- rous tissue, moderate lymphocyte and macrophage in- CASE 4 A 56-year-old medical practitioner was filtration, and a few plasma cells. admitted to hospital with a one-week history of dys- pnoea, precordial pain, and weight loss. A history CASE 5 A 60-year-old draughtsman was admitted to of dyspepsia in 1962 had been attributed to a duo- hospital for investigation of a 10-year history of denal ulcer but was mainly one of 'heartburn'. He dyspnoea, which was worse on exertion. He also gave no history of chest disease, allergy, arthritis, or complained of a non-productive cough and a nasal exposure to dusts or fumes. He had smoked 20 discharge. Heartburn and retrosternal pain had been cigarettes daily until five years previously. prominent for five years, although a barium meal one Examination showed finger clubbing and medium year before had revealed no abnormality. He gave scattered crepitations over both lower lobes. His no history of arthritis, acute chest disease, allergy, or chest radiograph (Fig. 3) suggested extensive fibrosis exposure to dusts or fumes, and had been a non- and gas transferase (CO uptake by steady state smoker for two years. method) was greatly diminished at 6 ml/mmHg/min Examination showed kyphoscoliosis, poor chest (normal 22-30 ml/mmHg/min). He was not anaemic. expansion, and scattered bilateral medium crackling Rheumatoid latex fixation test, LE cells in the blood, sounds. There was no finger clubbing. His chest radio- and examination of the sputum for acid-fast bacilli graph suggested widespread pulmonary fibrosis, Thorax: first published as 10.1136/thx.26.3.300 on 1 May 1971. Downloaded from Diffuse pulmonary fibrosis and hiatus hernia 303 .1...a.rij,-m FIG. 3. Chest radiograph oJ a 56-year-old man showing bilateral mottling, especially in the right. http://thorax.bmj.com/ maximal at the bases.