Hypertension and Renal Failure (Scleroderma Renal Crisis) in Progressive Systemic Sclerosis

Traub, Yehuda M.; Shapiro, Alvin P.; Rodnan, Gerald P.; Medsger, Thomas A.; McDonald, Robert H.; Steen, Virgina D.; Osial, Thaddeus A.; Tolchin, Sanford F.

doi:10.1097/00005792-198311000-00001

Cited by 221 publications

(106 citation statements)

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“…Other studies have shown that renal crisis affects 7-18% of SSc patients, largely those with diffuse skin involvement and then, usually within the first 5-6 years of SSc onset (1,3,5,7,25). Renal crisis occurred in only 2 of our patients (4%), both of whom had diffuse SSc: for 6 years in one and for 11 years in the other.…”

Section: Discussionmentioning

confidence: 48%

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Abnormalities of Renal Physiology in Systemic Sclerosis

Clements

Lachenbruch

Fürst

et al. 1994

Arthritis & Rheumatism

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Objective. To evaluate the prognostic value of measurements of renal plasma flow and circulating vasoactive hormones in patients with systemic sclerosis (SSc).Methods. Renal plasma flow (para-aminohippurate [PAH] clearance) and levels of selected circulating hormones (plasma renin activity [PRA] and serum aldosterone and catecholamines) were assessed in 57 SSc patients under the following conditions: at rest, in response to cold, while in upright position, and in response to sodium depletion. Survival was assessed 10.7 years after testing (20 patients had died).Results. Both unstimulated and stimulated PRA clearance rates were frequently elevated. Resting PAH clearances were usually abnormally low. PAH clearances and levels of aldosterone and catecholamines did not change during cold stimulation. Supine PRA levels

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Section: Discussionmentioning

confidence: 48%

“…The mean * SD of all the clearance periods at a given setting (usually [3][4][5] periods, each lasting 20-30 minutes) was calculated. The coefficient of variation for the PAH clearance was 15% (k7) in the recumbent position and 21% (k8) in the sitting position.…”

Section: Methodsmentioning

confidence: 99%

Abnormalities of Renal Physiology in Systemic Sclerosis

Clements

Lachenbruch

Fürst

et al. 1994

Arthritis & Rheumatism

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“…Although in some there was transient reduction in the mean pulmonary artery pressure and/or pulmonary vascular resistance, no oral therapy altered the downhill course. In our experience, PHT patients have survival rates that are even worse than those in patients with renal crisis (29). Peters-Golden et a1 (30) found a markedly decreased survival rate for patients with a DLco <40% of predicted normal, although the presence or absence of PHT and the cause of death were frequently not known.…”

Section: Discussionmentioning

confidence: 70%

Pulmonary hypertension in the crest syndrome variant of systemic sclerosis

Stupi

Steen

Owens

et al. 1986

Arthritis & Rheumatism

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334

176

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Pulmonary hypertension (PHT) occurred in 59 (9%) of 673 systemic sclerosis patients seen between 1963 and 1983. In 30 patients, all with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias), the pulmonary hypertension was isolated, i.e., independent of other pulmonary or cardiac conditions. In 20 patients, isolated PHT was demonstrated by cardiac catheterization. All had normal or only mildly decreased lung volumes, and mild or no pulmonary interstitial fibrosis on chest roentgenogram. In comparison with 287 CREST syndrome patients without PHT, these 20 patients had markedly reduced diffusing capacity for carbon monoxide (DLco) (mean 39% of predicted normal). In 6 patients, the low DLco antedated clinical evidence of PHT by 1–6 years. At autopsy there was marked intimal fibrosis with hyalinization and smooth muscle hypertrophy in the small‐ and medium‐sized arteries, without significant parenchymal fibrosis or inflammation. Patients with isolated PHT did not respond favorably to vasodilators and had a very poor prognosis, with a 2‐year cumulative survival rate of 40%. A DLco < 45% of predicted in the absence of pulmonary interstitial fibrosis may be an important predictor of the subsequent development of isolated PHT.

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“…In SRC patients, serum creatinine and blood urea nitrogen (BUN) are consistently elevated and are usually proportional to the severity of renal involvement (Steen, 2003). Renin blood levels are significantly elevated, especially in patients with malignant hypertension (Traub et al, 1983). Urinalysis may reveal microscopic hematuria and mild to moderate proteinuria (usually 0.5 to 2.5 grams per 24 hours) Steen, 2003).…”

Section: Clinical and Laboratory Featuresmentioning

confidence: 99%

“…The latter is often attributed to decreased perfusion due to chronic ischemic vasculopathy. Intermittent vasospasm of renal arteries, also known as renal Raynaud's phenomenon, has also been proposed as a potential contributor to SRC-induced kidney injury (Cannon, et al, 1974); Traub et al reported an increase in the frequency of SRC in the winter suggesting cold-induced vasoconstriction of renal arteries (Traub, et al, 1983). Vasospasm can also participate in renal injury in SSc patients even in the absence of SRC.…”

Section: Pathophysiologymentioning

confidence: 99%