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Renal Crisis (renal + crisis)

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Medical Sciences	100%

Selected Abstracts

IS SCLERODERMA RENAL CRISIS WITH ANTI-CENTROMERE ANTIBODY-POSITIVE LIMITED CUTANEOUS SYSTEMIC SCLEROSIS OVERLOOKED IN PATIENTS WITH HYPERTENSION AND/OR RENAL DYSFUNCTION?

NEPHROLOGY, Issue 2 2008
TOSHIRO SUGIMOTO
[source]

Recombinant human relaxin in the treatment of systemic sclerosis with diffuse cutaneous involvement: A randomized, double-blind, placebo-controlled trial,

ARTHRITIS & RHEUMATISM, Issue 4 2009
Dinesh Khanna
Objective A phase II randomized controlled trial of recombinant human relaxin suggested that a dosage of 25 ,g/kg/day was safe and clinically effective in improving skin disease and reducing functional disability in scleroderma (systemic sclerosis; SSc). We undertook a large randomized, double-blind, placebo-controlled clinical trial to compare placebo with 10 ,g/kg/day and 25 ,g/kg/day recombinant human relaxin, given for 24 weeks in patients with stable, diffuse, moderate-to-severe SSc. Methods Men and women ages 18,70 years with diffuse cutaneous SSc (dcSSc) were administered recombinant human relaxin (10 ,g/kg/day or 25 ,g/kg/day) or placebo for 24 weeks as a continuous subcutaneous infusion. There was a followup safety visit at week 28. Results The primary outcome measure, the modified Rodnan skin thickness score, was similar among the 3 groups at baseline and at weeks 4, 12, and 24. Secondary outcomes such as functional disability were similar in all 3 groups, while the forced vital capacity decreased significantly in the relaxin groups. The discontinuation of both doses of relaxin at week 24 led to statistically significant declines in creatinine clearance and serious renal adverse events (defined as doubling of serum creatinine, renal crisis, or grade 3 or 4 essential hypertension) in 7 patients who had received relaxin therapy but in none who had received placebo. Conclusion Recombinant relaxin was not significantly better than placebo in improving the total skin score or pulmonary function or in reducing functional disability in patients with dcSSc. In addition, relaxin was associated with serious renal adverse events, the majority of which occurred after stopping the infusion. If relaxin is used therapeutically for any conditions other than scleroderma, close monitoring of blood pressure and renal function must be performed. [source]

Anti,U3 RNP autoantibodies in systemic sclerosis

ARTHRITIS & RHEUMATISM, Issue 4 2009
Rohit Aggarwal
Objective To describe the classification, demographic and clinical features, and survival in anti,U3 RNP autoantibody,positive patients with systemic sclerosis (SSc). Methods Medical records of 108 anti,U3 RNP,positive and 2,471 anti,U3 RNP,negative SSc patients first evaluated during 1985,2003 were reviewed. Anti,U3 RNP antibody was detected by protein and RNA immunoprecipitation. Disease classification, demographic and clinical features, organ system involvement, and survival were compared between the 2 patient groups, by Student's t -test, chi-square analysis, and Mantel-Haenszel test. Results The anti,U3 RNP,positive group had a higher proportion of African American patients (27% versus 5%; P < 0.001) and male patients (29% versus 19%; P = 0.021), and was younger at the time of first physician diagnosis (mean age 42.8 years versus 47.4 years; P = 0.001). The 2 groups had similar proportions of patients with diffuse cutaneous involvement (47% and 45% in those with and those without anti,U3 RNP, respectively). However, among patients with diffuse cutaneous involvement, the mean maximum modified Rodnan skin score was significantly lower in the anti,U3 RNP group (22.3 versus 27.9; P < 0.001). Skeletal muscle involvement was more frequent in anti,U3 RNP,positive patients (25% versus 14%; P = 0.002), as was "intrinsic" pulmonary arterial hypertension (PAH) (31% versus 13%; P < 0.001). The frequency of gastrointestinal involvement, cardiac involvement, pulmonary fibrosis, and "renal crisis" did not differ significantly between the 2 groups. Survival was worse in the anti,U3 RNP,positive group (hazard ratio 1.38 [95% confidence interval 1.05,1.82]). PAH was the most common known cause of death in patients with anti,U3 RNP (30%, versus 10% in the anti,U3 RNP,negative group; P < 0.001). Conclusion The present findings demonstrate that the frequencies of African American race and male sex are greater among SSc patients with anti,U3 RNP antibody than those without, and the former group is younger at SSc diagnosis. Anti,U3 RNP,positive patients have more frequent skeletal muscle involvement and PAH, the latter being the most common cause of death. [source]

The clinical relevance of serum antinuclear antibodies in Japanese patients with systemic sclerosis

BRITISH JOURNAL OF DERMATOLOGY, Issue 3 2008
Y. Hamaguchi
Summary Background, Systemic sclerosis (SSc) is a connective tissue disorder with excessive fibrosis of the skin and various internal organs. Although SSc is a heterogeneous disease, it has been reported that the particular antinuclear antibodies (ANA) are often indicative of clinical features, disease course and overall severity. Objective, To clarify the association of clinical and prognostic features with serum ANA in Japanese patients with SSc. Methods, We studied 203 Japanese patients diagnosed with SSc, who visited our hospital during the period 1983,2005. Six SSc-related ANA were identified using indirect immunofluorescence, double immunodiffusion and immunoprecipitation assays. Results, Patients with SSc were classified into six ANA-based subgroups and a group without ANA. As expected, antitopoisomerase I antibody (Ab, n = 64), anti-RNA polymerases (RNAP) Ab (n = 12) and anti-U3 RNP Ab (n = 5) were associated with diffuse cutaneous SSc, whereas anticentromere Ab (ACA, n = 75), anti-Th/To Ab (n = 7) and anti-U1 RNP Ab (n = 10) were frequently detected in patients with limited cutaneous SSc. Clinical features of the ANA-negative group (n = 10) were heterogeneous. Consistent with previous findings in Caucasian and/or black African patients, antitopoisomerase I Ab was associated with the involvement of vascular and pulmonary fibrosis, leading to decreased survival rate. However, no patients with anti-RNAP Ab developed renal crisis and the frequency of isolated pulmonary hypertension in patients with ACA, anti-Th/To Ab or anti-U3 RNP Ab was similar to that in other ANA-based subgroups. Conclusion, These results indicate that the clinical relevance of SSc-related ANA in Japanese patients differs in some aspects from that in Caucasian and/or black African patients. [source]