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Comparative Study
. 2011 Jul;38(7):1317-25.
doi: 10.3899/jrheum.100956. Epub 2011 Jun 17.

Late-age onset systemic sclerosis

Rebecca L Manno  1 , Fredrick M Wigley, Allan C Gelber, Laura K Hummers
Affiliations
Comparative Study

Late-age onset systemic sclerosis

Rebecca L Manno et al. J Rheumatol. 2011 Jul.

Abstract

Objective: Although patients who develop systemic sclerosis (SSc) later in life (≥ 65 yrs) may express the entire clinical spectrum of disease, we hypothesize that patients with late-age onset SSc incur a different risk for specific organ manifestations of disease compared to those with early-age onset SSc.

Methods: In total, 2300 patients with SSc were evaluated between 1990 and 2009 and reviewed from a university-based scleroderma center cohort. Demographic profile, SSc subtype, autoantibody status, Medsger severity scores, pulmonary function tests, echocardiography, and right heart catheterization measures were compared between late-age onset vs younger-age onset patients with SSc.

Results: Overall, 2084 patients (91%) developed SSc prior to age 65, while 216 (9%) were ≥ 65 years. Late-age onset patients had a significantly higher proportion of anticentromere antibodies (42% vs 27%; p = 0.001) compared to early-age onset patients. Risk of pulmonary hypertension (OR 1.76, 95% CI 1.00, 3.12), muscle weakness (OR 1.85, 95% CI 1.30, 1.64), renal impairment (OR 2.83, 95% CI 1.98, 4.04), and cardiac disease (OR 2.69, 95% CI 1.92, 3.78) was greater among those with late-age onset SSc; although risk of digital ischemia (OR 0.64, 95% CI 0.47, 0.86) was reduced. The cumulative incidence of pulmonary hypertension at 5 years was greater among those with late-age onset SSc (9%) compared to those with early-age onset SSc (2.7%; log-rank, p < 0.001).

Conclusion: These findings suggest that older patients with SSc are at greater risk for pulmonary hypertension, renal impairment, cardiac disease, and muscle weakness. Awareness of the distinct risk for specific organ manifestations in SSc, in particular pulmonary hypertension, should guide the care of patients with SSc whose disease begins after age 65.

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Figures

Figure 1
Figure 1
Age of scleroderma onset as defined by age of first non-Raynaud's scleroderma symptom among 2300 patients evaluated at the Johns Hopkins Scleroderma Center from 1990–2009
Figure 2a
Figure 2a
Cumulative incidence of pulmonary hypertension, defined by right heart catheterization, by age of scleroderma onset among patients with two or more visits to the Johns Hopkins Scleroderma Center. P<0.001
Figure 2b
Figure 2b
Cumulative incidence of pulmonary hypertension, defined by right heart catheterization or echocardiogram, by age of scleroderma onset among patients with two or more visits to the Johns Hopkins Scleroderma Center. P<0.001
See this image and copyright information in PMC

References

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