Scleroderma Renal Crisis Patients Research Articles

Association of Race and Risk of Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis.

Scleroderma renal crisis (SRC) is a rare and severe manifestation of systemic sclerosis (SSc). Although it is well documented that Black patients with SSc have worse morbidity and mortality than non-Black patients, racial predilection for SRC is underreported. We examine the association of race and future development of SRC in an SSc cohort. Using the electronic health record of the US Military Health System, we conducted a comprehensive chart review of each patient with SSc from 2005 to 2016. The final study cohort was comprised of 31 SRC cases and 322 SSc without SRC controls. We conducted logistic regression of SRC as the outcome variable and race (Black versus non-Black) as the primary predictor variable, adjusted for age, estimated glomerular filtration rate, hypertension, and proteinuria at SSc diagnosis. Of 353 patients, 294 had identifiable race (79 Black, 215 non-Black). Thirteen of 79 Black patients (16.5%) versus 16 of 215 (7.4%) non-Black patients developed SRC (P=0.02). On adjusted analysis, Black patients had a significantly higher risk of developing SRC than non-Black patients (odds ratio 6.4 [95% confidence interval 1.3-31.2], P=0.02). Anti-Ro antibody was present in a higher proportion of Black SRC patients versus Black patients without SRC (45% versus 14%, P=0.01). Conversely, older age, thrombocytopenia, and anti-RNA polymerase III antibody at SSc diagnosis were significantly associated with future SRC in the non-Black cohort. Black race was independently associated with a higher risk of future SRC. Further studies are needed to elucidate the mechanisms that underlie this important association.

AB0653 The association of autoantibodies with morbidity and mortality of scleroderma renal crisis in Japan

BackgroundThe morbidity of scleroderma renal crisis (SRC) and autoantibodies in systemic sclerosis (SSc) vary by races and regions. Anti-RNA polymerase III is associated with SRC in America and European countries. However, the association of autoantibodies with SRC had not been elucidated in Japan.ObjectivesWe aimed to investigate the association of autoantibodies with morbidity and mortality of SRC in Japan.MethodsThe clinical characteristics and mortality of 330 patients with systemic sclerosis (SSc) at Kyoto University Hospital were retrospectively analyzed, focusing on anti-centromere, anti-RNA polymerase III, anti-topoisomerase I, and anti-U1-RNP. Logistic regression analyses were performed to examine the association of autoantibodies with the development and mortality of SRC. Kaplan-Meier survival analysis was performed comparing the groups classified by autoantibodies.ResultsAnti-centromere (n = 177/318, 56%), anti-topoisomerase I (n = 80/311, 26%), anti-RNA polymerase III (n = 27/204, 13%), and anti-U1-RNP (n = 24/305, 8%) were found in SSc patients (n = 330). SRC was observed in 24 out of 330 SSc patients, including anti-topoisomerase I (n = 12/24, 50%), anti-RNA polymerase III (n = 7/24, 29%), anti-U1-RNP (n = 5/24, 21%), and anti-centromere (n = 3/24, 13%). Anti-U1-RNP (odds ratio [95% confidence interval], 3.63 [1.11–10.2]), anti-RNA polymerase III (3.29 [1.16–8.70]), and anti-topoisomerase I (3.22 [1.37–7.57]) were associated with the development of SRC. All patients with SRC were treated with ACE inhibitors and the 1-year survival rate was 54%. Anti-topoisomerase I was associated with the 1-year mortality of SRC (6.00 [1.11–41.1]). When the survival rate was compared between the patients positive for anti-topoisomerase I (n=12) and negative for anti-topoisomerase I (n=12), the 1-year survival rate was 33% vs 75% (p=0.041), respectively (Figure 1A). Furthermore, the 1-year survival of anti-centromere (100%), anti-RNA polymerase III (83%), and others/not detected (50%) were shown in patients negative for anti-topoisomerase I (Figure 1B).Figure 1.Overall survival of patients with SRC according to the type of autoantibodies.(A) The survival rates in SRC patients who were positive for anti-topoisomerase I (solid line, n = 12) and those who were negative for anti-topoisomerase I (dotted line, n = 12).(B) SRC patients negative for anti-topoisomerase I were classified as patients with anti-RNA polymerase III (dotted line, n = 6), anti-centromere (broken line, n = 2), and others/not detected (chain line, n = 4).ConclusionSpecific SSc-related autoantibodies were associated with the morbidity and mortality in SRC.

Estimated glomerular filtration rate is a marker of mortality in the European Scleroderma Trials and Research Group (EUSTAR) database.

The study aim was to evaluate the estimated glomerular filtration rate (eGFR), its association with clinical disease and its predictive ability with respect to mortality in SSc patients from the European Scleroderma Trials and Research Group (EUSTAR) database. SSc patients from the EUSTAR database who had items required for the calculation of eGFR at a baseline visit and a second follow-up visit available were included. A cut-off eGFR value of 60 ml/min was chosen for all SSc patients, and 30 ml/min for those with scleroderma renal crisis (SRC). Cox regression and competing risk analysis were performed to evaluate the use of eGFR as a predictive factor of mortality. A total of 3650 SSc patients were included in this study. The median serum level of creatinine and the mean of eGFR were 0.8 mg/dl (interquartile range = 0.6-0.9) and 86.6 ± 23.7 ml/min, respectively. The eGFR was significantly lower in patients with pulmonary hypertension. Overall survival (OS) was significantly reduced in SSc patients with eGFR < 60 ml/min compared with patients with eGFR ≥ 60 ml/min [OS at 5 years 0.763 (95% CI: 0.700, 0.814) vs 0.903 (95% CI: 0.883, 0.919; P < 0.001)]. In multivariable analysis, OS was associated with male gender (P < 0.01), systolic pulmonary arterial pressure (sPAP) (P < 0.001) and eGFR (P < 0.001). The cumulative incidence of deaths due to SSc was associated with increased sPAP (P < 0.001) and reduced eGFR (P < 0.05). The OS at 5 years of 53 SRC patients was not significantly different between SSc patients with eGFR > 30 ml/min and those with eGFR <30 ml/min. eGFR represents a predictive risk factor for overall survival in SSc. The eGFR, however, does not represent a risk factor for death in SRC.

Scleroderma renal crisis: observations from the South Australian Scleroderma Register.

Scleroderma renal crisis (SRC) is a rare but feared complication with high morbidity and mortality. Its aetiopathogenesis is unclear. To investigate epidemiological, serologic and clinical features of all patients with SRC listed on the population-based South Australian Scleroderma Register and to examine possible factors in aetiopathogenesis. A case note review was performed on all SRC patients with relevant data extracted to determine incidence, clinical phenotype, presence of autoantibodies and survival. Possible precipitating and aetiopathogenic factors were also examined. Data from the South Australian Scleroderma Register and Australia Bureau of Statistics was sourced for comparative purposes. Over the 34-year period (1985-2018), 30 patients (21 females, 9 males) presented with SRC giving a South Australian mean annual incidence of 0.58/million/year (95% CI 0.39-0.89). Twenty-eight of these patients had diffuse cutaneous scleroderma and two with limited cutaneous scleroderma. The mean age at first symptom of scleroderma was 51.2 ± 15.9 (mean ± SD) years with SRC occurring 4.6 years later (median = 3.0 years, range 0.1-20 years). Possible precipitating factors for SRC included high dose steroids in five patients. Twelve patients were anti- RNA polymerase3 (RNAPol3) positive and two were anti-topoisomerase 1 (Topo1) positive. Renal outcome was poor with 13 patients requiring renal replacement therapy and two proceeding to renal transplantation. The mean age at death was 61.2 ± 11.6 years with SRC patient survival being significantly shorter than patients with diffuse scleroderma without renal involvement (P = 0.002). There was no significant difference in survival between the 1985-2002 and the 2003-2018 SRC cohorts (P = 0.2). Nailfold capillaroscopy performed in 10 patients revealed extensive microvascular damage with prominent capillary drop out. SRC is a rare occurrence with an incidence of 0.58/million/year in South Australia. This frequency has not changed over time. It continues to have a severe adverse outcome with frequent requirement for renal replacement therapy and poor survival. Nailfold capillaroscopy revealed evidence of extensive capillary damage. No improvement in survival was observed over the 34-year study period.

Clinical features and long-term outcomes of Chinese patients with scleroderma renal crisis.

To investigate the clinical features, treatments, and long-term outcomes of Chinese patients with scleroderma renal crisis (SRC). We retrospectively reviewed the clinical and laboratory data of 538 patients with systemic sclerosis (SSc) at our center from January 2009 to December 2016, including 29 SRC and 509 SSc without SRC patients. The treatments and long-term outcomes of patients with SRC were also retrospectively analyzed. The prevalence of SRC was 5.4% in our cohort. Male gender (odds ratio [OR] =4.194 [95% CI 1.494-11.773]), glucocorticoid exposure (OR=3.666 [1.484-9.056]), pericardial effusion (OR=11.180 [4.515-27.681]), and myocardial involvement (OR=7.958 [1.664-38.064]) were associated with an increased risk of development of SRC. Despite the wide use of angiotensin-converting enzyme inhibitors, the permanent dialysis rate of patients with SRC was 48.3%. Sixteen patients died during follow-up, and the estimated 1- and 5-year survival rates of patients with SRC were 62.1% and 47.3%, respectively. Withdrawal of dialysis (5 patients) and myocardial complications (3 patients) were the main causes of death in patients with SRC. Patients with serum creatinine level >500µmol/L before treatment (log rank test 5.051, P=0.025) and/or those who needed dialysis at the onset of SRC (log rank test 12.870, P<0.001) showed poorer prognosis. SRC is a rare but severe complication in patients with SSc. Male gender, glucocorticoid exposure, pericardial effusion, and myocardial involvement were risk factors in the development of SRC. Withdrawal of dialysis and myocardial complications were the main causes of death in Chinese patients with SRC.

Risk factors and outcome of Thai patients with scleroderma renal crisis: a disease duration-matched case control study.

Data regarding the prevalence, risk factors and outcome of scleroderma renal crisis (SRC) in Asian patients with systemic sclerosis (SSc) are limited. To determine the prevalence, risk factors and outcomes of SRC in Thai SSc patients. Medical records of all SSc patients seen at the Division of Rheumatology, Chiang Mai University, Thailand, from January 1990 to December 2015 were retrospectively reviewed. For each SRC case, a disease duration (±1 year) matched control (four SSc patient without SRC for each SRC patient) was identified. Of 608 SSc patients seen during the study period, 19 SRC cases were identified, resulting in an SRC prevalence of 3.13%, with 76 matched controls. Of the 19 cases, mean ± SD age and median (interquartile range 1-3) disease duration was 56.2 ± 13.8 years and 5 (3-22) months, respectively. Seventeen patients (89.5%) had diffuse cutaneous SSc. Twelve patients (63.2%) had hypertensive renal crisis and seven (36.8%) had normotensive renal crisis. Multivariate conditional logistic regression analyses showed that digital gangrene (adjusted odd ratio [AOR] 31.41, 95% CI = 1.16-852.23, P = 0.041), current prednisolone dose ≥ 15 mg/day (AOR 31.22, 95% CI = 1.59-613.85, P = 0.024), serum albumin < 3 mg/dL (AOR 7.97, 95% CI = 1.49-42.56, P = 0.015), and cardiac involvement (AOR = 6.62, 95% CI = 1.08-40.63, P = 0.041) were independent risk factors for SRC. Fifteen SRC patients (78.9%) required dialysis and 10 (52.6%) died. SRC was an uncommon complication in Thai patients with SSc, but is associated with high mortality. Digital gangrene, current prednisolone dose ≥ 15 mg/day, serum albumin < 3 mg/dL and cardiac involvement were independent risk factors for SRC.

Analysis of complement biomarkers in systemic sclerosis indicates a distinct pattern in scleroderma renal crisis.

BackgroundThe complement system has been implicated in pathogenesis of systemic sclerosis (SSc). The goal of the present study was to evaluate improved complement biomarkers in SSc.MethodsThe presence of C4d, reflecting activation of the classical/lectin pathways, C3bBbP corresponding to activation of the alternative pathway, and soluble terminal complement complexes (all complement pathways), was measured in plasma samples by enzyme-linked immunosorbent assay and correlated to clinical parameters. The study included 81 patients with limited cutaneous SSc and 41 with diffuse cutaneous SSc, as well as 47 matched healthy controls and 81 patients with rheumatoid arthritis, 22 with psoriatic arthritis and 20 with ankylosing spondylitis. Skin and kidney biopsies of selected patients were stained to detect deposited C3b as a marker of local complement activation.ResultsBiomarkers of activation of all complement pathways were increased in SSc compared with healthy controls and were similar to those in other rheumatic diseases. When patients with SSc were divided into subgroups, a distinct pattern of complement markers was observed in individuals with scleroderma renal crisis (SRC). By functional assay, we confirmed a significant decrease in complement haemolytic activity in SRC vs. non-SRC patients, indicating complement consumption. Further, we detected glomerular deposits of C3b in some patients with SRC.ConclusionsThe data indicate that complement activation is an important feature of SRC.

A retrospective clinical analysis of 16 patients with scleroderma renal crisis

To analyze the clinical features, laboratory tests, treatments and outcome of patients with scleroderma renal crisis (SRC). We retrospectively reviewed the clinical and laboratory data of 16 patients with scleroderma renal crisis in Peking Union Medical College Hospital from May 2004 to May 2013. The treatment and outcome of SRC patients were also retrospectively analyzed. There were a total of 16 SRC patients including 5 male patients and 11 females. The median age at SRC onset was (49.9 ± 12.3) years. It usually took 3.2 years from the diagnosis of systemic sclerosis (SSc) to SRC attack. Ten SRC patients belonged to diffuse cutaneous systemic sclerosis (dcSSc), and 6 patients were limited cutaneous systemic sclerosis (lcSSc). Among SRC patients, 16/16 were negative of anti-centromere antibodies (ACAs). All these 16 patients had hypertension and renal insufficiency, including 8 dialysis dependent after the onset of SRC and 7 with thrombotic microangiopathy. There were 3 patients receiving renal biopsy. The pathological findings were mainly summarized as intimal thickening and stenosis of renal arterioles. Among 13 patients with long-term followed-up, 11 patients received angiotensin converting enzyme inhibitors (ACEI), 5 patients died, 2 patients were dialysis dependent. Only 1 patient stopped dialysis after the combination treatment of ACEI and endothelin receptor antagonist. Another 5 patients didn't need dialysis. SRC usually occurred at the early course of SSc. dcSSc was more frequent than lcSSc. ACAs were rarely found in SRC patients. The immediate and sufficient use of ACEIs was still the cornerstone of SRC treatment. Future studies are needed to evaluate the efficacy of endothelin receptor antagonist in the treatment of SRC.

Scleroderma renal crisis: a retrospective multicentre study on 91 patients and 427 controls

Scleroderma renal crisis (SRC) is a severe manifestation of SSc, whose prognosis remains severe, despite treatment with angiotensin-converting-enzyme inhibitor and dialysis. This study was undertaken to describe SRC characteristics, prognosis and outcome, and evaluate the responsibility of CSs in its occurrence. Analysis concerned 91 SSc patients with SRC who were compared with 427 non-SRC-SSc patients taken as controls. Among the 91 SRC patients, 71 (78.0%) had high blood pressure, 53 (58.2%) hypertensive encephalopathy and 51 (56.0%) thrombotic microangiopathy; 64 (70.3%) had received CSs before or concomitantly with SRC vs 156 (36.5%) non-SRC-SSc patients (P < 0.001). Treated SRC patients also received more prednisone 29.3 (28.4) vs 3.6 (9.9) mg than controls (P < 0.001). SRC clinical outcomes were poor: 49 (53.8%) patients required dialysis, which was definitive for 38. Thirty-seven (40.7%) SRC patients died vs 10.8% of the controls (P < 0.001). Death was most frequent among dialysed patients who never recovered renal function (22 vs 2) and 13 never-dialysed SRC patients died. Although SRC prognosis has improved markedly, SRC remains a severe manifestation of SSc, despite treatment with angiotensin-converting enzyme inhibitor and dialysis. CSs contributed significantly to SRC occurrence.

Scleroderma renal crisis in a Swedish systemic sclerosis cohort: survival, renal outcome, and RNA polymerase III antibodies as a risk factor

Objectives: To study survival, renal outcome, and RNA polymerase III antibodies (RNAP Abs) as a risk factor for scleroderma renal crisis (SRC) in a Swedish cohort of systemic sclerosis (SSc) patients.Methods: SRC was diagnosed in 16 SSc patients during the period from 1982 to 2010. For comparison, 112 (seven for each SRC patient) SSc patients without SRC were included. RNAP Abs were detected by a fully automated fluoroenzymeimmunoassay (EliA). Values greater than 15 μg/L were considered positive. Frozen serum samples from the time of diagnosis of SSc were used.Results: The 5- and 10-year survival rates were, respectively, 58% and 40% for SRC patients and 90% and 76% for patients without SRC (p < 0.001). The odds ratio (OR) for mortality was 4.39 [95% confidence interval (CI) 2.10–9.16, p < 0.001] in patients with SRC compared to those without SRC. Renal outcome was good in three patients. Haemodialysis was started in 10 patients and peritoneal dialysis in three. Renal function improved in three patients and dialysis was terminated. Four patients underwent renal transplantation. Seven SRC patients and nine without SRC were positive for RNAP Abs. Anti-RNAP Abs was a strong predictor of SRC. The sensitivity and specificity for development of SRC were 0.44 and 0.92, respectively. The OR for development of SRC was 8.90 (95% CI 2.68–29.6, p = 0.001) in RNAP-positive patients.Conclusions: RNAP positivity is a strong risk factor for SRC. Renal outcome was variable and survival is still notably decreased.

アンジオテンシンII受容体拮抗薬(losartan)が有効と考えられた強皮症腎クリーゼの1例

A 67-year-old man with a one-and-a half-year history of Raynaud's phenomenon was admitted to our hospital for progressive dyspnea occurring over the previous two weeks. Physical examination revealed a blood pressure of 200/124 mmHg, and slightly tight and smooth skin of the fingers, hands and forearms. Laboratory evaluation included serum creatinine of 5.42 mg/dl, plasma renin activity > 20 ng/ml/hr, and antinuclear antibody with a titer of 1 : 1,280. Renal biopsy was performed and the histopathological findings showed that some glomeruli exhibited ischemic retraction with wrinkling of the basement membranes, and that one arteriole exhibited significant intimal hyperplasia with luminal stenosis. These findings were compatible with scleroderma renal crisis (SRC). On the 5th day, serum creatinine had risen to 9.16 mg/dl, and he required temporary hemodialysis therapy. After the administration of captopril was started, his blood pressure fell to 160/86 mmHg and serum creatinine was reduced to 5.12 mg/dl. On the 9th day, he exhibited skin eruptions, and captopril was discontinued accordingly and temocapril started. Because of continued eruptions, temocapril was replaced by losartan. His blood pressure was controlled easily and his serum creatinine level reduced steadily. One year after the start of losartan, serum creatinine was 2.25 mg/dl and blood pressure was 130/82 mmHg. SRC is a life-threatening manifestation of systemic sclerosis. In the late 1970s, angiotensin converting enzyme (ACE) inhibitor was introduced and has dramatically improved the outcome in SRC patients. As ACE inhibitors act mainly on hyperreninemic renal vasoconstrictive hypertension in SRC, we would expect losartan, a selective antagonist of angiotensin receptor subtype 1, to be interchangeable with ACE inhibitors in SRC. In 1997, Caskey and colleagues reported the failure of losartan to control hypertension in a patient of SRC, and the reason has remained unclear. We report here, a case of SRC whose blood pressure was controlled successfully and his renal failure reversed by the administration of losartan.

Association of RNA Polymerase III Antibodies with Scleroderma Renal Crisis

To the Editor: The study by Cordullo, et al investigated the prevalence of systemic sclerosis (SSc)-related autoantibodies among SSc patients with scleroderma renal crisis (SRC) in Italy. The authors report that the majority of the SRC patients had anti-topoisomerase I (anti-topo I) antibody (30/46, 65%), whereas a minority of patients (7/46, 15%) had anti-RNA polymerase III (RNAP)1. We reviewed cases of SRC in the Scleroderma Family Registry and DNA Repository (Registry) in the USA, which consisted of 1029 patients with SSc at the time of analysis. All patients fulfilled American College of Rheumatology preliminary classification criteria2 or had at least 3 of the 5 features of CREST (calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia); the diagnosis was verified by medical record review. The Registry database included demographic features, clinical disease characteristics, including extent of skin involvement, and autoantibody … Address correspondence to Dr. Nguyen. E-mail: Binh.Nguyen{at}uth.tmc.edu

Scleroderma Renal Crisis: A Pathology Perspective

Scleroderma renal crisis (SRC) is an infrequent but serious complication of systemic sclerosis (SSc). It is associated with increased vascular permeability, activation of coagulation cascade, and renin secretion, which may lead to the acute renal failure typically associated with accelerated hypertension. The histologic picture of SRC is that of a thrombotic microangiopathy process with prominent small vessel involvement manifesting as myxoid intimal changes, thrombi, onion skin lesions, and/or fibrointimal sclerosis. Renal biopsies play an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in SSc patients, helping to predict the clinical outcome and optimizing patient management. Kidney transplantation may be the only treatment option available for a subset of SRC patients who develop end-stage renal failure despite aggressive angiotensin-converting enzyme inhibitor therapy. However, the posttransplant outcome for SSc patients is currently suboptimal compared to the general renal transplant population.

Scleroderma Renal Crisis: A Rare Complication of Systemic Sclerosis with Poor prognosis-experience in a Medical Center in Central Taiwan

Objective: Scleroderma renal crisis (SRC) is the most life-threatening complication of systemic sclerosis (SSc), which is characterized by malignant hypertension and rapidly progressive renal failure. In this study, we investigate the incidence, clinical features, treatment and outcome of our SRC patients. Patients and Methods: We reviewed the records of 161 patients with SSc, seen from January 1991 to December 2005 in the Department of Rheumatology, Taichung Veterans General Hospital, Taiwan. Tests for anti-nuclear antibodies (ANA) in sera were screened against Hep-2 cells by indirect immunofluorescence assay. Quantification of autoantibodies to extractable nuclear antigen, including anti-Scl-70 antibody (Scl-70) and anti-centromere antibody, were tested with the AtheNa Multi-Lyte ANA test system. Data collected from the records included gender, age at onset, age at diagnosis, clinical manifestations, laboratory data, treatment and outcome. Fisher's exact test was used to compare differences in categorical variables. Results: Seventy-eight (48.4%) patients were limited type SSc and 83 (51.6%) patients were diffuse type SSc. Four (2.5%) patients of a total of 161 patients had SRC. All of our SRC patients were diffuse type with positive Scl-70. SRC occurred at 37.7±55.72 months (range from 1-120 months) after the diagnosis of SSc. The duration between Raynaud's phenomenon to disease onset was 3.75 ± 2.06 months. There were significant difference at myocardial involvement (p＜0.05) and pericardial effusion (p＜0.05) between the SRC and Non-SRC diffuse SSc patients. Two patients died within four months after the SRC, one patient required permanent dialysis and one patient partly regained renal function. Conclusion: SRC is a rare complication in Taiwanese SSc patients and has a poor prognosis. Whether early administration of angiotensin converting enzyme inhibitor might prevent or ameliorate the onset of SRC needs further investigation.

Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis.

To determine whether the initiation of corticosteroids or other types of therapy affects the development of scleroderma renal crisis (SRC). Using a case-control study, 110 patients with systemic sclerosis who developed SRC between 1981 and 1993 were closely matched with controls on sex, race, age, disease duration, skin score, levels of creatine phosphokinase, and presence of tendon friction rubs. Corticosteroid use was determined prior to the onset of SRC in cases or prior to the first visit in controls. Cases were compared with matched controls using McNemar's matched-pair analysis and conditional logistic regression analysis. The effects of other drugs, including D-penicillamine, nonsteroidal antiinflammatory drugs (NSAIDs), calcium channel blockers, and angiotensin-converting enzyme (ACE) inhibitors, were also evaluated. In the 6 months prior to SRC onset or to the first visit, high-dose corticosteroids (> or =15 mg/day prednisone or equivalent) were administered significantly more frequently in SRC patients (36%) than in the controls (12%) (McNemar's odds ratio 4.37, 95% confidence interval 2.03-9.43, P < 0.0001). New use of low-dose steroids, continuous use of any steroid dose, NSAIDs, calcium channel blockers, and ACE inhibitors were not associated with an increased risk of SRC. Antecedent D-penicillamine therapy may have been protective against the development of SRC in controls. This retrospective case-control study has shown a significant association between antecedent high-dose corticosteroid therapy and the development of SRC. These results should discourage the use of high-dose corticosteroids in patients with early diffuse scleroderma who are at increased risk of developing SRC.

Scleroderma Renal Crisis Analysis of Prevalence and Outcome in a Large Italian Series

During a 30-year period, 323 SSc patients were admitted to a tertiary center specialized in connective tissue diseases. Among them 13 (4|X%) developed scleroderma renal crisis (SRC). These 13 SRC patients with respect to the remaining SSc patients were older, were more often affected by diffuse cutaneous systemic sclerosis (dcSSc), and had slightly higher blood pressure and more frequent heart involvement at initial presentation. Among the 13 SRC patients, an older age, a shorter disease duration, and a higher peak serum creatinine correlated with a poor outcome.SRC in Italian patients showed a lower prevalence (4|X%) than in many earlier reports. Mean survival in SRC patients was 6 years. This tended to be less than the 16.5 years for all others, but this trend was not statistically significant at least for those admitted in the post-captopril era.From a clinical point of view, the higher prevalence of later SRC in dcSSc patients presenting with mild arterial hypertension and or heart involvement is worth noting. Many patients were asymptomatic and were detected only on a routine visit. Such patients must be carefully followed to detect any early finding of SRC.