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Purpose. In lcSSc patients, PAH may occur with minor or no evidence of pulmonary fibrosis or heart disease. Its frequency ranges from 8-40%, depending on selection of patients and its prognosis is poor with a 2 year survival rate of 40% from diagnosis. We retrospectively analyzed a large series of lcSSc patients to ascertain characteristics that predict the development of IPAH and to identify risk factors for this complication.
Patients and Methods. Patients with lcSSc without evidence of primary or secondary PAH at first visit (1972-2002) were followed for the occurrence of IPAH. We excluded patients who developed secondary PAH and those lost to follow-up after 2002. All records were reviewed for IPAH proved by echocardiogram (mean pulmonary artery pressure [PAP] > 40 mmHg at rest), right heart catheterization (mean PAP > 25 mmHg at rest) and/or autopsy. Where those procedures were not performed IPAH diagnosis was based on clinical and other laboratory findings. The date of onset of IPAH was the first symptom attributable to PAH or laboratory confirmation of PAH, whichever occurred first. Statistical analysis included Chi2 and student’s t test, survival analysis (IPAH as endpoint) and Cox proportional hazard ratio for multivariate analysis.
Results. We compared the 45 (7%) lcSSc patients who developed IPAH during follow-up with the 607 who did not. Ninety-eight percent of the IPAH patients were Caucasian and 87% female (NS vs. no-IPAH patients). The mean age at onset of SSc was 41 years and the mean age at onset of IPAH was 64 years. Thus, IPAH was identified a mean of 20 years after the onset of lcSSc; at that time point the likelihood of developing IPAH was 5% for the whole group. Patients with IPAH had higher risk of mortality. We divided the patients according to the age at onset of Raynaud phenomenon into three groups: <35 years (early Raynaud onset, ERO), 35-54 years (intermediate Raynaud onset, IRO) and 55 years or more (late Raynaud onset, LRO). The probability of developing IPAH progressively increased in ERO, IRO and LRO groups; 4%, 11% and 19% respectively (p<0.001). Univariate analysis showed that digital tip scars, ulcers and gangrene, history of scleroderma renal crisis (SRC) and lower carbon monoxide diffusing capacity (predicted DLCO ≤ 50%) were associated with the development of IPAH. Use of calcium channel blockers (CCB) at any time during the disease was protective. On multivariate analysis only age at onset of Raynaud phenomenon (p<0.0001), history of SRC (p=0.02) and low DLCO (p=0.02) remained significant and CCB showed a trend.
Conclusions. LcSSc patients with onset of Raynaud phenomenon at an older age, low DLCO and/or history of SRC should be regarded as having an increased risk of developing IPAH and should be monitored closely for this complication. It is possible that calcium channel blocking drugs reduce the likelihood of IPAH in lcSSc patients.
Patients and Methods. Patients with lcSSc without evidence of primary or secondary PAH at first visit (1972-2002) were followed for the occurrence of IPAH. We excluded patients who developed secondary PAH and those lost to follow-up after 2002. All records were reviewed for IPAH proved by echocardiogram (mean pulmonary artery pressure [PAP] > 40 mmHg at rest), right heart catheterization (mean PAP > 25 mmHg at rest) and/or autopsy. Where those procedures were not performed IPAH diagnosis was based on clinical and other laboratory findings. The date of onset of IPAH was the first symptom attributable to PAH or laboratory confirmation of PAH, whichever occurred first. Statistical analysis included Chi2 and student’s t test, survival analysis (IPAH as endpoint) and Cox proportional hazard ratio for multivariate analysis.
Results. We compared the 45 (7%) lcSSc patients who developed IPAH during follow-up with the 607 who did not. Ninety-eight percent of the IPAH patients were Caucasian and 87% female (NS vs. no-IPAH patients). The mean age at onset of SSc was 41 years and the mean age at onset of IPAH was 64 years. Thus, IPAH was identified a mean of 20 years after the onset of lcSSc; at that time point the likelihood of developing IPAH was 5% for the whole group. Patients with IPAH had higher risk of mortality. We divided the patients according to the age at onset of Raynaud phenomenon into three groups: <35 years (early Raynaud onset, ERO), 35-54 years (intermediate Raynaud onset, IRO) and 55 years or more (late Raynaud onset, LRO). The probability of developing IPAH progressively increased in ERO, IRO and LRO groups; 4%, 11% and 19% respectively (p<0.001). Univariate analysis showed that digital tip scars, ulcers and gangrene, history of scleroderma renal crisis (SRC) and lower carbon monoxide diffusing capacity (predicted DLCO ≤ 50%) were associated with the development of IPAH. Use of calcium channel blockers (CCB) at any time during the disease was protective. On multivariate analysis only age at onset of Raynaud phenomenon (p<0.0001), history of SRC (p=0.02) and low DLCO (p=0.02) remained significant and CCB showed a trend.
Conclusions. LcSSc patients with onset of Raynaud phenomenon at an older age, low DLCO and/or history of SRC should be regarded as having an increased risk of developing IPAH and should be monitored closely for this complication. It is possible that calcium channel blocking drugs reduce the likelihood of IPAH in lcSSc patients.
T. Rodriguez-Reyna, None.