Pulmonary Hypertension in the CREST Syndrome Variant of Progressive Systemic Sclerosis (Scleroderma)

Salerni, Rosemarie

doi:10.7326/0003-4819-86-4-394

Cited by 251 publications

(78 citation statements)

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“…Serum complement levels are generally normal. In immunohistologic studies, investigators have demonstrated immunoglobulin deposition in the glomeruli and the interlobular arteries of patients with PSS and renal involvement (15), as well as in the pulmonary vasculature of some patients (16). However, numerous studies (3,17) of the skin of PSS patients have failed to identify such deposits at either the dermal-epidermal junction or in the dermal vasculature.…”

Section: Discussionmentioning

confidence: 99%

“…Single serum samples from 92 patients with PSS were examined; the group included 68 women and 24 men, ranging in age at study from 18 to 85 years (mean 48.3). Forty-eight of these individuals (32 women, 16 phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) variant of PSS (3). The median duration of disease was 3.4 years (1.3 years in the patients with diffuse scleroderma and 13.4 years in those with the CREST syndrome).…”

Section: Methodsmentioning

confidence: 99%

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Immune complexes in progressive systemic sclerosis (scleroderma)

Seibold

Medsger

Winkelstein

et al. 1982

Arthritis & Rheumatism

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Serum immune complexes were measured in 92 patients with progressive systemic sclerosis, and elevated levels were found as follows: Raji cell assay 72% (59% after pronase treatment of Raji cells), agarose gel electrophoresis 52%, and Clq binding 24%. Fortythree (47%) had abnormal results on two or more of these tests, but only 17 (18%) had normal results by all three assays. Computer-assisted analysis of immune complex results and extensive clinical and laboratory data compiled on these patients revealed that the patients with abnormal Raji cell assays more often had diffuse scleroderma, tendon friction rubs, and positive serum antinuclear antibody tests than did patients with negative results on Raji cell assays. Individuals with immune complexes detected by Clq binding had evi- dence of pulmonary involvement and positive serum rheumatoid factor more frequently than did patients whose Clq tests were negative.The etiology of progressive systemic sclerosis (PSS, scleroderma) is unknown. Any hypothesis concerning the pathogenesis of this disease must account for both the prominence of vascular lesions in involved tissues (1,2) and the high frequency of abnormal serologic and cellular immune reactions (3). Initiation or mediation of vascular damage by circulating immune complexes might satisfy these requirements. In reports to date on immune complexes in PSS, investigators have studied small groups of patients and used a variety of immune complex assays, with contradictory results (4-8).In the present study, sera of 92 unselected patients with PSS were examined for the presence of immune complexes by three techniques: the Raji cell radioimmunoassay (RCA) (9), the Clq binding assay (ClqBA) (lo), and pattern recognition on agarose gel electrophoresis (AGE) (1 1). Correlations with clinical and other laboratory features of PSS were sought by computer analysis of an extensive database compiled on these patients in our center as part of the American Rheumatism Association Medical Information System (ARAMIS) (12). PATIENTS AND METHODSSelection of patients. Single serum samples from 92 patients with PSS were examined; the group included 68 women and 24 men, ranging in age at study from 18 to 85 years (mean 48.3). Forty-eight of these individuals (32 women, 16 men) had diffuse scleroderma and 44 (36 women, 8 men) had the CREST syndrome (calcinosis, Raynaud's

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Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Immune complexes in progressive systemic sclerosis (scleroderma)

Seibold

Medsger

Winkelstein

et al. 1982

Arthritis & Rheumatism

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“…The most common type of involvement is cor pulmonale secondary to pulmonary hypertension. Patients with the CREST syndrome may have either a mild and prolonged course (7)(8)(9) or a progressive course with pulmonary and GI involvement (9). Death usually results from pulmonary hypertension and cor pulmonale (9).…”

Section: Discussionmentioning

confidence: 99%

Calcific constrictive pericarditis. A rare complication of CREST syndrome

Panchal

Adams

Hsieh

1996

Arthritis & Rheumatism

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This report describes a patient with limited cutaneous scleroderma in whom calcific constrictive pericarditis developed. This complication of limited cutaneous scleroderma has not been reported previously.Pericarditis is a common feature of systemic sclerosis. Constrictive pericarditis is rare, however, and calcific constrictive pericarditis as a complication of CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) (limited cutaneous scleroderma) has not been described. We report herein a case of limited cutaneous scleroderma with severe refractory heart failure secondary to calcific constrictive pericarditis. CASE REPORTThe patient, a 70-year-old man, was admitted to the hospital with progressive shortness of breath, paroxysmal nocturnal dyspnea, and orthopnea of several days' duration. He had a history of congestive heart failure (CHF) and pacemaker insertion for bradycardia, but his condition had been stable prior to this episode.The patient had been diagnosed as having limited cutaneous scleroderma (CREST syndrome) in 1989, with Raynaud's phenomenon, sclerodactyly, telangiectasias on the face, lips, and hands, and extensive calcific deposits on the elbows and over the left knee, intermittently complicated by cellulitis. indwelling catheter for an atonic bladder, hypothyroidism, hepatomegaly , and history of cerebrovascular accident. His current medications included allopurinol, furosemide, KCl, levothyroxine, warfarin, and ranitidine.On examination he was found to be in moderate respiratory distress. His pulse rate was 66 beats/ minute, and the blood pressure was 160190 mm Hg without pulsus paradoxus. Neck veins were distended up to the angle of the jaw at 45". Auscultation of the heart revealed a II/IV systolic ejection murmur and an audible S3. Lung examination showed wet rales halfway up both lung fields. The liver was enlarged to 13 cm. Physical and radiologic examination revealed calcinosis of the knees, elbows, and fingers (Figure l). He had numerous telangiectasias. He had indurated, thickened skin over both shins with 1 + edema, but the remainder of the skin, including that on the hands, was normal.During this admission the patient was treated initially with high-dose furosemide (160 mg twice daily with repeated intravenous boluses). Later, bumetanide and metolazone were tried, with some success.Laboratory studies revealed a blood urea nitrogen level of 43 mg/dl, a creatinine level of 1.9 mgldl, a hemoglobin value of 10.0 gm%. He was hypoxic, with an arterial blood gas pH of 7.47 (Po2 64). The erythrocyte sedimentation rate was 15 mm/hour. Liver enzymes were mildly elevated. Protein electrophoresis results were within normal limits. Antinuclear antibody was positive at a titer of 1 : 1,280, with a centromere pattern. Purified protein derivative was negative. Chest radiography revealed an enlarged heart with left ventricular predominance, pulmonary vascular redistribution, periventricular infiltrates, and extensive pericardial calcification (Figures ...

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“…Although myocardial involvement, hypertension, and pulmonary fibrosis can also occur in scleroderma generally, and CREST in particular, pulmonary hypertension can occur independently (Botstein and LeRoy, 1981;Bulkley et al, 1976;Gaffrey et al, 1982;Salerni et al, 1977;Smith er al., 1979;Trell and Lindstrom, 1971;Young and Mark, 1978). Pulmonary hypertension frequently leads to progressive right heart failure and death (Salemi et a]., 1977).…”

Section: Discussionmentioning

confidence: 99%

Unloading the right ventricle in the crest syndrome variant of progressive systemic sclerosis (scleroderma)

Morrison

Goldman

Alepa

1984

Clinical Cardiology

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Summary: A patient with severe pulmonary hypertension secondary to the CREST syndrome variant of scleroderma is described. Acute reductions in both pulmonary artery pressure and total pulmonary resistance were seen with nifedipine and oxygen administration. Reductions in resistance were maintained for over one month with this combination. These results raise the possibility that some of the pulmonary hypertension seen in this condition is reversible.

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Pulmonary Hypertension in the CREST Syndrome Variant of Progressive Systemic Sclerosis (Scleroderma)

Cited by 251 publications

References 28 publications

Immune complexes in progressive systemic sclerosis (scleroderma)

Immune complexes in progressive systemic sclerosis (scleroderma)

Calcific constrictive pericarditis. A rare complication of CREST syndrome

Unloading the right ventricle in the crest syndrome variant of progressive systemic sclerosis (scleroderma)

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