Progressive generalized scleroderma: respiratory failure from primary chest wall involvement.

A 73-year-old woman presented with an eight-month history of swelling of the right leg. The calf and ankle were found normal, but there was non-pitting swelling of the thigh of "woody" consistency. Both flanks were firm with cutaneous thickening. There were no other abnormal physical findings. A cutaneous biopsy confirmed the diagnosis of scleroderma. The chest radiograph was normal and initial lung function tests within normal limits-FEV1 1x60 1 (1x61 1), FVC 1-75 1 (2-06 1), FEV1/FVC 91% (72%), TLC 4-31 1 (4,2 1), VC 1 99 1 (2-27 1), RV 2-32 1 (188 1), transfer factor 5 46 mM.min'l.kPa'" (7,92 mM.min'1. kPa'1), normal values being shown in brackets. The electrocardiogram showed an old inferior myocardial infarction. The ESR was 19 mm in one hour and antinuclear factor positive. Three months later sclerodermatous changes were noted in the skin of the left leg with more widespread involvement of the anterior abdominal wall extending up to the lower costal margins. Chest radiograph and lung function tests were again within normal limits and barium swallow showed no evidence of oesophageal involvement. Treatment was started with oral penicillamine 250 mg bd. Despite three months of therapy, however, sclerodermatous changes had extended to involve the entire chest wall, and the left inguinal region. Neck movement was restricted and the arms could not be raised above the head. Oral prednisolone 40 mg daily was added to the treatment regime, but with little objective improvement. A short course of azathioprine was given, but discontinued because of a marked leucopenia and thrombocytopenia. Eight months after presentation the patient became somnolent with shallow respiration. Arterial blood gas estimations showed hypoxia and hypercapnia with P°2 5*0 kPa, Pco2 93 kPa, and H+ 62 nM. Arterial oxygen saturation, as determined by ear oximeter, was 5242%. Forced hyperventilation, however, reversed these changes (fig 1) to Po2 12,1 kPa, Pco2 5-1 kPa, H+ 44 nM, and an arterial oxygen saturation of 90%. Chest radiograph was again normal and the alveolararterial oxygen difference within normal limits (2-28 kPa). Spirometry showed a reduction in FEV1 to 0-90 1 and FVC to 1-00 1. A doxapram infusion was started to stimulate respiratory effects but had minimal effect. Continuous nocturnal recording of arterial oxygen saturation by ear oximetry showed values as low as 44% during doxapram infusion, but rising to 95 % on waking. The most powerful stimulus to respiration was the use of the bedpan. Twenty-four per cent oxygen by face mask was used with some benefit on oxygen tension without increasing hypercapnia. Finally, both medroxyprogesterone 300 mg daily and dexamphetamine daily were commenced, but with little effect. She became increasingly somnolent, comatose, and died within a week in respiratory failure.