TY - JOUR
T1 - The 15% Rule in Scleroderma
T2 - The Frequency of Severe Organ Complications in Systemic Sclerosis. A Systematic Review
AU - Muangchan, Chayawee
AU - Markland, J.
AU - Robinson, D.
AU - Jones, N.
AU - Khalidi, N.
AU - Docherty, P.
AU - Kaminska, E.
AU - Masetto, A.
AU - Sutton, E.
AU - Mathieu, J. P.
AU - Hudson, M.
AU - Ligier, S.
AU - Grodzicky, T.
AU - LeClercq, S.
AU - Thorne, C.
AU - Fritzler, M.
AU - Baron, Murray
AU - Pope, Janet
PY - 2013/9
Y1 - 2013/9
N2 - Objective. The prevalence of organ complications in scleroderma (systemic sclerosis; SSc) varies by definition used. This study was done to determine the frequency of several features of SSc. Methods. A search of Medline-Ovid/Embase, PubMed, and Scopus databases from 1980 to November 30, 2011, was conducted to identify relevant articles with at least 50 patients with SSc extracting prevalence of each organ complication. Study quality was assessed using the STROBE (Strengthening The Reporting of OBservational studies in Epidemiology) checklist. Pooled prevalence was calculated using the random effects method. Heterogeneity was quantified using I2. Results. A total of 5916 articles were identified (913 from Medline-Ovid/Embase, 1009 from PubMed, and 3994 from Scopus); 5665 were excluded, leaving 251 articles for full-text review, with 69 included. Where available, frequencies were also included from the Canadian Scleroderma Research Group. Many severe complications in SSc occur about 15% of the time, including cardiac involvement (15%, 95% CI 6-24), diastolic dysfunction (16%, 95% CI 14-17), estimated pulmonary artery pressure > 40 mm Hg (18%, 95% CI 14-21), pulmonary arterial hypertension by right heart catheterization (15%, 95% CI 12-17), forced vital capacity (FVC) < 70% predicted (15%, 95% CI 12-17), FVC < 80% predicted (17%, 95% CI 12-21), myositis (13%, 95% CI 10-17), inflammatory arthritis (12%, 95% CI 9-16), Sjögren overlap (13%, 95% CI 10-16), and digital ulcers (DU; 15%, 95% CI 10-20); and 15% of DU have complications (amputations 12%, 95% CI 8-16, and hospitalizations 13%, 95% CI 6-21). Scleroderma renal crisis is uncommon but occurs in almost 15% (12%, 95% CI 5-19) of cases of disseminated cutaneous SSc. There is no 15% rule within skin and gastrointestinal tract for SSc. Conclusion. The "15%" rule for frequency of significant organ involvement in SSc is helpful. The Journal of Rheumatology
AB - Objective. The prevalence of organ complications in scleroderma (systemic sclerosis; SSc) varies by definition used. This study was done to determine the frequency of several features of SSc. Methods. A search of Medline-Ovid/Embase, PubMed, and Scopus databases from 1980 to November 30, 2011, was conducted to identify relevant articles with at least 50 patients with SSc extracting prevalence of each organ complication. Study quality was assessed using the STROBE (Strengthening The Reporting of OBservational studies in Epidemiology) checklist. Pooled prevalence was calculated using the random effects method. Heterogeneity was quantified using I2. Results. A total of 5916 articles were identified (913 from Medline-Ovid/Embase, 1009 from PubMed, and 3994 from Scopus); 5665 were excluded, leaving 251 articles for full-text review, with 69 included. Where available, frequencies were also included from the Canadian Scleroderma Research Group. Many severe complications in SSc occur about 15% of the time, including cardiac involvement (15%, 95% CI 6-24), diastolic dysfunction (16%, 95% CI 14-17), estimated pulmonary artery pressure > 40 mm Hg (18%, 95% CI 14-21), pulmonary arterial hypertension by right heart catheterization (15%, 95% CI 12-17), forced vital capacity (FVC) < 70% predicted (15%, 95% CI 12-17), FVC < 80% predicted (17%, 95% CI 12-21), myositis (13%, 95% CI 10-17), inflammatory arthritis (12%, 95% CI 9-16), Sjögren overlap (13%, 95% CI 10-16), and digital ulcers (DU; 15%, 95% CI 10-20); and 15% of DU have complications (amputations 12%, 95% CI 8-16, and hospitalizations 13%, 95% CI 6-21). Scleroderma renal crisis is uncommon but occurs in almost 15% (12%, 95% CI 5-19) of cases of disseminated cutaneous SSc. There is no 15% rule within skin and gastrointestinal tract for SSc. Conclusion. The "15%" rule for frequency of significant organ involvement in SSc is helpful. The Journal of Rheumatology
KW - Digital ulcer
KW - Myopathy
KW - Pulmonary artery hypertension
KW - Pulmonary fibrosis
KW - Renal crisis
KW - Scleroderma
UR - http://www.scopus.com/inward/record.url?scp=84883431993&partnerID=8YFLogxK
U2 - 10.3899/jrheum.121380
DO - 10.3899/jrheum.121380
M3 - Review article
C2 - 23858045
AN - SCOPUS:84883431993
SN - 0315-162X
VL - 40
SP - 1545
EP - 1556
JO - Journal of Rheumatology
JF - Journal of Rheumatology
IS - 9
ER -
