Pulmonary Fibrosis in Scleroderma

Abstract

THE following case of pulmonary fibrosis occurring with scleroderma is recorded mainly on account of its rarity and general interest, and also in order to emphasize the fact that scleroderma is not a condition confined to tlie skin and subcutaneous tissues. Rather it may involve the connective tissues throughout the entire body. CASE REPORT,This patient, a man, aged 56, was admitted to Charing Cross Hospital on 12 July 1948, complaining of shortness of breath on exertion, with occasional precordial pain at rest, for the last three years.Until three years ago he was a very active man, but then found that on exertion he was short of breath ; this became worse, until, on his admission, the exertion of sitting up in bed would precipitate an attack of acute respiratory distress lasting two or three minutes. At about the same time he first had pain, when sitting down, more especially in the late aftemoon, over the lower left chest. The pain did not radiate down his arm. It was not associated with headache or with palpitations. He attended the National Heart Hospital, where he was seen by Dr. William Evans, who found no cardiac disorder.During the following year the above symptoms continued, and he found that he became very sleepy during the day. Two years ago he noticed that he Avaa losing weight. He had been 13 st. 5 lb., and gradually declined to his present weight of S St. 8 lb. His own practitioner saw him at this time, and noticed clubbing of the fingers, and blueness of the fingers in the cold weather. There was a slight morning cough, with a small amount of sputum. At this stage a diagnosis was made of left ventricular failure due to coronary sclerosis, thought to be associated with changes in the lungs secondary to a rise in the venous pressure. He was treated with mersalyl.The clubbing of the fingers became more pronounced, and was followed by clubbing of the toes. In the last year his appetite has failed.Six months ago his doctor noticed that the skin over the backs of the hands, forehead and cheeks appeared tight and thickened. There is no history of preceding oedema or inflammatory change. The rest of the skin was normal.The past history included diphtheria as a boy, and cholecystotomy in 1943. He worked in a Welsh coal mine for one month as a boy, doing clerical work imderground. For the rest of his life he had worked in a shop, dealing with ladies' wear. The family history contained nothing of relevance.On examination there was dyspnoea on the slightest exertion. He could hold his breath for 10 seconds only. There were fine rales at both bases, respiration being almost entirely abdominal, and vocal fremitus more marked on the right side. Finger and toe clubbing was marked. The feet and hands were cold and white. There was a fine telangiectasia over the upper part of the back. No abnormalities were noted in the cardiovascular system. The pulse was 80 per minute and the blood pressure was 100/70. The temperature was normal throughout the period of observation in hospital.

Pulmonary Manifestations in the Diffuse Collagen Diseases

“…Pulmonary manifestations in diffuse collagen disorders are not uncommon and changes varying from pleural effusions, partial consolidation, widespread reticulation, miliary mottling to chronic fibrosis and sclero-cystic lung disease have been described in the rheumatic diseases (Cheadle, 1888;von Glahn and Pappenheimer, 1926;Masson, Riopelle, and Martin, 1937;Hadfield, 1938;Gouley, 1938;Harkavy, 1941Harkavy, , 1943Rakov and Taylor, 1942;Baggenstoss and Rosenberg, 1943;Gregory and Rich, 1946;Jensen, 1946;Klemperer, 1948;Ellman, 1947;Ellman and Ball, 1948; van Wijk, 1948;and Lees, 1952); in polyarteritis nodosa (Weir, 1939;Elkeles, 1944;Miller and Daley, 1946;and Bergstrand, 1946); in disseminated lupus erythematosus (Tumulty and Harvey, 1949); and in scleroderma (Finlay, 1891;Kraus, 1924;Matsui, 1924;Murphy, Krainin, and Gerson, 1941 ;Linenthal and Talkov, 1941 ;Weiss, Stead, Warren, and Bailey, 1943;Jackman, 1943;Bevans, 1945;Getzowa, 1945;Goetz, 1945;Dostrovsky, 1947;Baehr and Pollack, 1947;Lloyd and Tonkin, 1948;McMichael, 1948 ;Wigley, Edmunds, and Bradley, 1949;Church and Ellis, 1950;…”