Pulmonary hypertension in systemic sclerosis.
Authors: E Trell, C Lindstrom
Journal: Annals of the Rheumatic Diseases (1971)

Pulmonary fibrosis is common in systemic sclerosis, and may lead to cor pulmonale.Changes in the pulmonary arterial system also occur and may cause progressive pulmonary hypertension and right heart failure.A case is reported in detail below, with eight other cases which came to necropsy. Case reportA woman born in 1912 (Case 8 in the necropsy series) had an unremarkable family history.In 1940, she first noted blanching and cyanosis of the fingers on exposure to cold.Since 1960, small, hard, subcutaneous nodules developed at different locations on her body.Early in 1968, progressive effort dypsnoea and anginal chest pains started insidiously, and treatment with digitalis, furosemide, and potassium was instituted.In September, 1968, she was admitted to the Medical Clinic for heart catheterization.Anticoagulants, reser- pine, and theophylline were added to her regimen, but her condition gradually deteriorated, with fatigue, dyspnoea at rest, anginal pains, ankle oedema, cyanosis, and syncopal attacks.She died on January 26, 1969. Physical examinationThe patient was thin and emaciated.The skin showed marked greyish-brown hyperpigmentation but no teleangiectasia.The joints were normal but the glistening skin of the fingers, hands, and forearms was adherent to the indurated underlying tissue, restricting movement in the finger joints.Atrophy and tapering of the digits were noted, as were hard subcutaneous nodules, particularly over both patellae.Other physical findings included cyanosis, moderate ankle and crural oedema, distended neck-veins and slight hepatomegaly.The breath-sounds were normal.Over the heart, there was a right ventricular lift, and the pulmonic component of the widely split second sound was accentuated.At the apex and in the fourth intercostal space near the left sternal border a grade 2-3/6 pansystolic, low-frequent murmur was present.Consecutive laboratory findings Arterial P02 72-61 mm.Hg and PCO2 26-29 mm.Hg.Haemoglobin 17 -6-14-5 g./100 ml.RBC 5 * 4-51 million/mm.3,WBC 4,500-8,600/mm.3,Differential count normal.Platelets 170,000-120,000/mm.3Serum bilirubin 1-8-2-4 mg./100 ml. with a positive direct reaction.SGPT 35-100 Karmen units; SGOT 19-100 Karmen units; serum alkaline phosphatase normal; serum y-glutamyl transpeptidase 127-98 units (normal 15-80); serum lactate dehydrogenase 585 Wroblewski units with slight elevations of the iso-enzyme fractions LDH1 and LDH2 and, less pronounced, LDH3.Thymol extinction normal.Agarose gel electrophoresis and quantitative plasma protein determinations showed a slight inflammatory reaction and normal values of IgG, IgA, and IgM.Serum cryoglobulin negative.Erythrocyte sedimentation rate 2-1 mm./lst hour.Extensive serological tests revealed positive Waaler- Rose (titre 1/64) and F II A (titre 1/160) reactions, and a weakly positive antinuclear factor reaction (titre 1/20).LE-cells were not found.Detailed coagulation analyses exhibited increased platelet adhesiveness and fibrinolysis inhibitor activity, and decreased vessel-wall fibrinolytic activity, but were otherwise normal. Further investigationsOrdinary chest x rays (Fig. 1, opposite) revealed no sign of pulmonary fibrosis.The heart measured 1,060-950 ml./640-610 ml. per M.2 BSA and indicated right ven- tricular enlargement.X-ray investigations of the hands and knees (Fig. 2, opposite) exhibited subcutaneous calcifications (Thibierge-Weissenbach). Angiocardiography (Fig. 3, overleaf) showed marked right ventricular dilatation and hypertrophy, and an extremely slow contrast passage through the lungs.The pulmonary arteries were dilated centrally, but from the secondary divisions there was a marked narrowing of their calibre.Spirometry in July and September, 1969, showed ventilatory capacity 83-80 per cent. of calculated normal