title: Late-age onset systemic sclerosis.
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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA.
authors.forename: Rebecca L
authors.initials: RL
authors.lastname: Manno
authors.forename: Fredrick M
authors.initials: FM
authors.lastname: Wigley
authors.forename: Allan C
authors.initials: AC
authors.lastname: Gelber
authors.forename: Laura K
authors.initials: LK
authors.lastname: Hummers
mesh_terms: D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D015331:Cohort Studies; D004452:Echocardiography; D005260:Female; D006331:Heart Diseases / Q000453:epidemiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology*; D007674:Kidney Diseases / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000453:epidemiology*; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications* / Q000453:epidemiology*; D012720:Severity of Illness Index
chemical_list: D001323:Autoantibodies