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• W2442412638 abstract "Case ReportsPulmonary Hemorrhage Complicatinga Case of Systemic Sclerosis Hasan O. Naddaf and MRCP(UK) Suliman R. Al BallaFRCP(C) Hasan O. Naddaf Address reprint requests and correspondence to Dr. Naddaf: RO. Box 17401, Riyadh 11484, Saudi Arabia. From the Division of Rheumatology, College of Medicine, King Saud University, Riyadh Search for more papers by this author and Suliman R. Al Balla From the Division of Rheumatology, College of Medicine, King Saud University, Riyadh Search for more papers by this author Published Online:1 Jan 1996https://doi.org/10.5144/0256-4947.1996.71SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutAbstractDiffuse pulmonary hemorrhage is a serious condition that may complicate many connective tissue diseases, most commonly systemic lupus erythematosus. However, there are very few case reports of pulmonary hemorrhage complicating systemic sclerosis. Here we report a patient with systemic sclerosis who presented with sudden hemoptysis, a drop in hemoglobin, hypoxia and bilateral alveolar infiltrates. He responded rapidly to prednisolone and plasmapheresis.CASE REPORTA 35-year-old engineer was diagnosed in 1990 as having systemic sclerosis on the basis of Raynaud's phenomenon, sclerodactyly, tight skin over the face and hands, a peaked nose, limited mouth opening to 3 cm and positive antinuclear antibody (ANA nucleolar pattern).In 1993, the patient presented with progressive dysphagia, nasal speech and marked weight loss (15 kg in five months). Physical examination showed features of systemic sclerosis, marked proximal muscle weakness, and normal cranial nerve examination; all other systemic examinations were normal. Investigations for underlying infection, malignancy, malabsorption and endocrine diseases were all normal. A barium meal and esophageal motility studies disclosed esophageal reflux and motility disturbance consistent with progressive systemic sclerosis.Creatinine phosphokinase enzyme (CPK) level was very high at 3000 U/L (N=30 to 210); electromyography (EMG) was consistent with polymyositis. This diagnosis was confirmed by a muscle biopsy. A chest x-ray showed a very small bilateral basal infiltrate (Figure 1). The pulmonary function tests showed mild restrictive pattern.Figure 1. Chest x-ray of the patient two months prior to onset of pulmonary hemorrhage, showing very little bilateral basilar infiltrate.Download FigureBecause of polymyositis and severe constitutional symptoms, the patient was started on prednisolone 1.5 mg/kg/day. He showed very good improvement with regard to muscle weakness and appetite; he gained 7 kg within two months. His CPK returned to normal.Three months later the patient presented with a 12-hour history of cough and hemoptysis. There was no sputum production, fever or chest pain. On examination, he was afebrile (36.7 °C) and there were a few bilateral basilar crepitations. His hemoglobin was 15 g/dL, WBC 5.6×l09/L. Arterial blood gases showed severe hypoxia, PO2 was 51 mmHg, PCO2 was 37 mmHg, pH was 7.44 and oxygen saturation was 88%. His hypoxia did not improve with 50% oxygen. A chest x-ray showed diffuse bilateral alveolar infiltrates (Figure 2). The patient was started on oxygen and broad spectrum antibiotics. Twelve hours later, his hemoptysis increased and his hemoglobin dropped by 5 g over the next two days. Investigations showed a normal prothrombin time, partial thromboplastin time, liver function tests, urea and electrolytes. Antinuclear antibody was positive with a titer of 1:1280 with a nucleolar pattern; double-strand DNA and rheumatoid factor were negative. Antibasement membrane antibody (ABMA) and antineutrophil cytoplasmic antibody (ANCA) were negative and ribonucleoprotein (RNP) antibody was negative.Figure 2. Chest x-ray showing bilateral diffuse alveolar infiltrate.Download FigureSeptic screen was negative, including several blood cultures, sputum culture, mycoplasma pneumonia titer, cold agglutinins, legionella titer, sputum viral cultures and fungal cultures. Bronchoscopy was refused by the patient. Urine microscopy, culture and 24-hour urine collection for protein were all normal. An echocardiogram showed normal left ventricular function.With this clinical picture of sudden hemoptysis, a drop in hemoglobin of 5 g, bilateral alveolar shadowing, no fever, no leukocytosis, and negative cultures, the patient was managed for pulmonary hemorrhage with an increasing dose of prednisolone to 90 mg and plasmapheresis. Five days after the onset of the symptoms, the patient improved, hemoptysis stopped, oxygenation improved and the chest x-ray showed the infiltrate had almost disappeared (Figure 3).Figure 3. Chest x-ray five days after onset with almost complete clearing of alveolar infiltrate.Download FigureDISCUSSIONPulmonary hemorrhage is a serious condition. It may be idiopathic or associated with a number of connective tissue diseases. Idiopathic pulmonary hemorrhage is usually diagnosed in the absence of renal disease, vasculitis, left side heart failure or antiglomerular basement membrane antibody (AGBMA).1Idiopathic pulmonary hemorrhage was described with a number of connective tissue diseases, such as rheumatoid arthritis,2,3 systemic lupus erythematosus,4 Wegner's granulomatosis,5 and Goodpasture's syndrome;6 however, there are very few case reports of pulmonary hemorrhage associated with systemic sclerosis. J. Kellenbach et al. reported the first case in 19777 of a female with progressive systemic sclerosis and pulmonary hemorrhage who responded to 40 mg of prednisolone. J. L. Alvarez Vega et al. reported another case in 1992;8 their patients, however, developed focal necrotizing glomerulonephritis later on. This patient also responded to corticosteroid treatment.The mechanism of pulmonary hemorrhage is uncertain. It may be a distinct pathological entity due to abnormal pulmonary alveolar epithelial growth and function resulting in alveolar capillary hemorrhage.9 On the other hand, it may be immunological in nature, with involvement of pulmonary capillaries as part of a multisystem involvement similar to Goodpasture's syndrome, mediated by antiglomerular basement membrane antibody.10Corticosteroids were helpful in the previously reported cases of systemic sclerosis and pulmonary hemorrhage; plasmapheresis is also helpful in patients with Goodpasture's syndrome as well as pulmonary hemorrhage associated with systemic lupus erythematosus.Our patient presented with sudden hemoptysis, a drop in hemoglobin of 5 g, and bilateral alveolar infiltrates with rapid clearing in five days. He had no evidence of infection, vasculitis, or renal disease with a negative antiglomerular basement membrane antibody.The patient fit the clinical criteria of pulmonary hemorrhage. With the few cases reported previously of pulmonary hemorrhage associated with systemic sclerosis, we think systemic sclerosis should be kept in mind as a possible cause of idiopathic pulmonary hemorrhage.ARTICLE REFERENCES:Thomas HM, Irwin RS. Classification of diffuse intrapulmonary hemorrhage . Chest. 1975; 68:483–4. Google ScholarKarlish AJ. Idiopathic pulmonary hemosidrosis with unusual features . Proceedings of the Royal Society of Medicine. 1962; 55:223–5. Google ScholarOgnibene AJ, Dito WR. Rheumatoid disease with unusual pulmonary manifestations . Arch Int Med. 1965; 116:567–72. Google ScholarByrd RB, Trunk G. Systemic lupus erythematosus presenting as pulmonary hemosiderosis . Chest. 1973; 64:128–9. Google ScholarO'Donohue WJ. Idiopathic pulmonary hemosiderosis with manifestations of multiple connective tissue and immune disorders. Treatment with cyclophosphamide . Am Rev Resp Dis. 1974; 109:473–9. Google ScholarClinicopathological conference. Proliferative glomerulonephritis and pulmonary hemorrhage . Am J Med. 1973; 55:199–210. Google ScholarKallenbach J, Prinsloo I, Zwi S. Progressive systemic sclerosis complicated by diffuse pulmonary hemorrhage . Thorax. 1977; 32:767–70. Google ScholarAlvarez JL. et al.. Pulmonary hemorrhage and focal necrotizing glomerulonephritis in a case of systemic sclerosis . Clin Rheumatol. 1992; 11:116–9. Google ScholarSoergel KH, Sommers SC. Idiopathic pulmonary hemosidrosis and related syndromes . Am J Med. 1962; 32:499–511. Google ScholarElder JL, Kirke GM, Smith WG. Idiopathic pulmonary hemosidrosis and the Goodpasture syndrome . Br Med J. 1965; 2:1152–5. Google Scholar Previous article Next article FiguresReferencesRelatedDetails Volume 16, Issue 1January 1996 Metrics History Accepted7 May 1995Published online1 January 1996 InformationCopyright © 1996, Annals of Saudi MedicinePDF download" @default.
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