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Results for “Scleroderma, Diffuse”

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PubMed1987

Mast cell numbers in diffuse scleroderma.

Numbers of mast cells were quantitated in the lesions of diffuse scleroderma and morphea. Mast cells increased and then decreased in number in the papillary dermis of diffuse scleroderma. No significant change of mast cell numbers was noted in the reticular dermis. Mast cells increased in the papillary dermis with fine collagen bundles (grade 2 skin of scleroderma) and decreased in the papillary dermis with homogeneous collagen bundles (grade 3 skin of scleroderma). The total number of cells increased in the papillary dermis of grade 1 and 2 skin of scleroderma and decreased in the grade 3 skin of scleroderma. In morphea a reduced number of mast cells was noted in grade 3 lesions. It is suggested that mast cells play an important role in fibrotic process of scleroderma skin.

Cell Count↗
PubMed1984

Physiologic abnormalities of cardiac function in progressive systemic sclerosis with diffuse scleroderma.

To investigate cardiopulmonary function in progressive systemic sclerosis with diffuse scleroderma, we studied 26 patients with maximal exercise and redistribution thallium scans, rest and exercise radionuclide ventriculography, pulmonary-function testing, and chest roentgenography. Although only 6 patients had clinical evidence of cardiac involvement, 20 had abnormal thallium scans, including 10 with reversible exercise-induced defects and 18 with fixed defects (8 had both). Seven of the 10 patients who had exercise-induced defects and underwent cardiac catheterization had normal coronary angiograms. Mean resting left ventricular ejection fraction and mean resting right ventricular ejection fraction were lower in patients with post-exercise left ventricular thallium defect scores above the median (59 +/- 13 per cent vs. 69 +/- 6 per cent [P less than 0.025], and 36 +/- 12 per cent vs. 47 +/- 7 per cent [P less than 0.025], respectively). We conclude that in progressive systemic sclerosis with diffuse scleroderma, abnormalities of myocardial perfusion are common and appear to be due to a disturbance of the myocardial microcirculation. Both right and left ventricular dysfunction appear to be related to this circulatory disturbance, suggesting ischemically mediated injury.

Adult↗
PubMed1983

Pulmonary function in progressive systemic sclerosis. Comparison of CREST syndrome variant with diffuse scleroderma.

The pulmonary function and chest roentgenograms were evaluated in 88 patients with the CREST syndrome variant of progressive systemic sclerosis (PSS or scleroderma). Seventy-two percent of the patients had abnormal pulmonary function. An isolated decrease in diffusing capacity was the most common abnormality noted, followed by restrictive abnormalities and airway obstruction. Chest roentgenograms revealed interstitial infiltrates consistent with pulmonary fibrosis in 33 percent. When compared to a contemporaneous group of 77 patients with PSS and diffuse scleroderma, patients with the CREST syndrome had similar abnormalities on pulmonary function testing and chest roentgenogram. However, patients with the CREST syndrome had a lower mean diffusing capacity despite a higher mean vital capacity; this combination of findings suggests primary pulmonary vascular disease. Calcified granulomata were identified significantly more often in PSS-CREST patients, while superior rib notching occurred exclusively in patients with PSS and diffuse scleroderma. The CREST variant of PSS is associated with frequent roentgenographic and pulmonary function abnormalities similar to those seen in PSS with diffuse scleroderma.

Adult↗
PubMed2001

Pilot study of anti-thymocyte globulin plus mycophenolate mofetil in recent-onset diffuse scleroderma.

OBJECTIVE: To assess the safety and efficacy of anti-thymocyte globulin (ATG) followed by mycophenolate mofetil (MMF) in the treatment of diffuse scleroderma. METHODS: A pilot study of 13 patients with recent-onset diffuse scleroderma was carried out. Patients received ATG for 5 days, followed by MMF for 12 months. We recorded adverse events, scleroderma skin score, hand contractures, EuroQol score, scleroderma functional assessment, pulmonary function studies, echocardiogram and plasma creatinine concentration. RESULTS: Mean skin score decreased during the study from 28 at baseline to 17 after 12 months of MMF (P<0.01). Hand contractures worsened during the study. Mean measurements of systemic disease remained stable. One patient died after a scleroderma renal crisis. Five patients developed serum sickness after ATG treatment, but this was controlled by corticosteroid therapy. MMF therapy was well tolerated. CONCLUSION: ATG and MMF appear safe in scleroderma. The improvement in skin score and the apparent stability of systemic disease during the study period suggest that controlled studies of these agents are justified.

Adult↗
PubMed1980

Reversible acute renal faiure in diffuse scleroderma.

A case of severe hypertension and acute renal failure developing in a 56 year old woman affected by diffuse scleroderma is reported. The patient was treated by peritoneal dialysis, antihypertensive agents and plasma infusions, which led to the correction of arterial hypertension and partial recovery of renal function. This report emphasizes the possibility that the correction of malignant hypertension may prevent progressive renal falure in some patients affected by diffuse scleroderma.

Acute Kidney Injury↗
PubMed1979

[Carpal tunnel syndrome in diffuse scleroderma].

A 61 year old female patient with diffuse scleroderma, complicated by carpal tunnel syndrome, is reported. The coexistence of carpal tunnel syndrome with dermatomyositis and lupus erythematodes is reviewed.

Carpal Tunnel Syndrome↗
PubMed2001

Pseudohypertension in a patient with diffuse scleroderma.

Pseudohypertension is the artifactual elevation of blood pressure that occurs secondary to noncompressible blood vessels. It has been described in patients with uremia, diabetes mellitus, and severe atherosclerosis. If unrecognized, the condition may lead to inappropriate and potentially harmful therapy. We report a case of pseudohypertension in a 65-year-old man with diffuse scleroderma. His blood pressure as assessed by conventional sphygmomanometry was at least 240/135 to 145 mm Hg. Intra-arterial blood pressure was found to be 107/52 mm Hg. The severe rise in blood pressure as measured by sphygmomanometry led to the concern of scleroderma renal crisis and potentially harmful therapy. Intra-arterial pressure monitoring confirmed the presence of pseudohypertension, however. This is the first reported case of pseudohypertension in a patient with diffuse scleroderma.

Aged↗
PubMed1996

Prevalence of pulmonary hypertension in limited and diffuse scleroderma.

STUDY OBJECTIVES: To characterize the prevalence of undiagnosed pulmonary hypertension in patients with limited and diffuse scleroderma. DESIGN: Prospective cross-sectional study. SETTING: University-based outpatient clinic. PATIENTS: Thirty-four consecutive patients with limited (n = 29) or diffuse (n = 5) scleroderma but without the clinical diagnosis of pulmonary hypertension. MEASUREMENTS AND RESULTS: All patients had 12-lead ECGs and two-dimensional and Doppler echocardiograms. The pulmonary artery systolic pressure (PAs) was calculated as the sum of the Doppler transtricuspid pressure gradient and the right atrial pressure as estimated by the caval respiratory index. Thirty-three patients (97%) had adequate spectral signals of tricuspid regurgitation. The velocity of tricuspid regurgitation ranged from 1.6 to 4.5 m/s. The calculated PAs ranged from 15 to 95 (mean +/- SD = 30 +/- 14 mm Hg). Twelve patients (35% of the total cohort) had pulmonary hypertension defined as PAs of 30 mm Hg or greater. CONCLUSIONS: Undiagnosed elevation of PAs is common in patients with scleroderma. Noninvasive assessment of PAs can be performed accurately in most patients independent of clinical signs of pulmonary hypertension. If successful treatment strategies are identified, it may be possible to identify patients early in the development of pulmonary hypertension and intervene before significant end-organ damage occurs.

Adult↗
PubMed1994

Autonomic dysfunction in diffuse scleroderma vs CREST: an assessment by computerized heart rate variability.

OBJECTIVE: Autonomic dysfunction has been described in scleroderma. Heart rate variability gives information about sympathetic-parasympathetic autonomic balance, therefore it can provide insight into which division of the system is most affected. Our purpose was to determine whether the neural control of heart rate in patients with CREST syndrome differed from that in patients with diffuse scleroderma. Since low heart rate variability in some diseases is associated with an increased risk for malignant ventricular arrhythmias, assessment of patients with scleroderma with this technique might establish a marker of adverse outcome. METHODS: A short time analysis of heart rate variability was performed in 9 patients with diffuse scleroderma, 8 with CREST and 17 age/sex matched healthy controls. RESULTS: An abnormal heart rate variability was found in patients with CREST: There was a reduced standard deviation of the mean of beat-to-beat R-R intervals and a low power spectrum density in both the 0.05 to 0.15 Hz band and the 0.15 to 0.35 Hz band, a significant reduction in their total power (p < 0.05), a reduction in the high frequency to total power ratio (p < 0.05), and a reduction in the ratio between high and low frequency components of their spectrum. CONCLUSION: It is suggested that patients with CREST have a decreased parasympathetic control of heart rate, significantly different from those with diffuse disease. Whether this finding is pertinent to the differences between these subsets of patients remains to be established.

Adult↗
PubMed2003

Diffuse scleroderma occurring after the use of paclitaxel for ovarian cancer.

Acase of diffuse scleroderma in a 56-year-old woman who received paclitaxel for the treatment of a metastatic ovarian cancer is presented. The clinical cutaneous alterations, as well as the capillaroscopic and histological findings, were indistinguishable from those encountered in definite systemic sclerosis (SSc). In contrast to SSc, Raynaud's phenomenon and cutaneous calcinosis were absent and antinuclear antibodies were negative. The temporal relationship between the onset of skin involvement and administration of the drug may indicate an effect of paclitaxel.

Antineoplastic Agents, Phytogenic↗
PubMed2000

Severe organ involvement in systemic sclerosis with diffuse scleroderma.

OBJECTIVE: To determine the natural history and timing of severe involvement of the kidney, heart, lung, gastrointestinal (GI) tract, and skin in patients with systemic sclerosis (SSc) and diffuse cutaneous involvement. METHODS: This study used the Pittsburgh Scleroderma Databank and included patients with diffuse scleroderma who were seen between January 1, 1972 and December 31, 1995. Patients had frequent follow-ups, and a 95% accountability for these patients was maintained. Severe organ involvement was defined as the presence of any of the following: 1) in the kidney, scleroderma "renal crisis"; 2) in the heart, cardiomyopathy, symptomatic pericarditis, or an arrhythmia requiring treatment; 3) in the lung, pulmonary fibrosis on chest radiograph and a forced vital capacity of <55% of predicted; 4) in the GI tract, malabsorption, repeated episodes of pseudoobstruction, or severe problems requiring hyperalimentation; and 5) in the skin, a modified Rodnan skin score >40. The timing from disease onset to survival for each case of severe organ involvement was determined. RESULTS: Of the 953 patients with diffuse scleroderma, kidney involvement developed in 177 (19%), heart involvement in 143 (15%), lung involvement in 151 (16%), GI tract involvement in 74 (8%), and skin involvement in 233 (24%). Severe skin and kidney involvement occurred during the first 3 years in 70% of those who ever developed these problems throughout a mean of 10 years of followup. Severe heart, lung, and GI tract involvement developed during the first 3 years in 45-55% of those who were ever affected. The survival of patients with severe organ involvement was poor. The 9-year cumulative survival rate of all patients with severe organ involvement was 38%, compared with 72% in patients without such involvement (P < 0.0001). CONCLUSION: This study demonstrates that severe organ involvement in SSc patients with diffuse scleroderma most often occurs early in the course of the disease. Survival for patients with severe organ involvement is markedly reduced. Patients should therefore be monitored very closely during the first 3 years of disease for signs and symptoms that may signal the subsequent development of severe organ damage. Potential disease-modifying therapies must be initiated early to modify the natural history of SSc and to improve survival. Patients who survive the first few years without developing severe organ involvement are less likely to develop such life-threatening involvement later in the disease course.

Female↗
PubMed1986

Pharmacodynamic effect of dipyridamole on thallium-201 myocardial perfusion in progressive systemic sclerosis with diffuse scleroderma.

We evaluated the effect of dipyridamole on thallium-201 myocardial perfusion in 23 patients with progressive systemic sclerosis (PSS) with diffuse scleroderma. Thallium-201 single photon emission computed tomography (SPECT) was performed at rest and after coronary artery vasodilatation with intravenous dipyridamole (0.14 mg/kg/min for four minutes). The left myocardium was divided into nine segments; each segment was graded as 2.0, 1.5, 1.0, 0.5, 0 (zero represents no activity). Dipyridamole significantly improved resting thallium-201 myocardial perfusion: the mean (SD) number of segments with thallium defects decreased from 6.0 (2.1) at rest to 4.1 (2.5) after dipyridamole (p less than 0.0001); the mean (SD) score in segments with resting defects increased from 0.92 (0.24) at rest to 1.13 (0.38) after dipyridamole (p less than 0.0001); the mean (SD) global score per patient increased from 10.2 (1.8) at rest to 11.4 (2.1) after dipyridamole (p less than 0.02); the global score increased by at least 2.0 in 12 patients and worsened by at least 2.0 in three patients only (p = 0.05). The results of this acute study suggest that some drugs with potent vasodilator activity on small coronary arteries may be beneficial in the treatment of PSS patients with thallium-201 myocardial perfusion abnormalities.

Adult↗