Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose:
Renal disease is a common complication of scleroderma (SSc). Isolated reduction in glomerular filtration rate (GFR), a marker of impaired renal function, can occur in patients with normal serum creatinine. A recent single-center study of patients with SSc and pulmonary arterial hypertension (SSc-PAH) suggests that an estimated GFR (eGFR) < 60 ml/min/1.73m2at baseline occurs in over 40% of patients and portends a three-fold increased risk of death in this population. Therefore, we sought to determine the prevalence and clinical correlates of renal dysfunction in a large, multi-center observational cohort using the PHAROS (Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma) registry.
Methods: : We identified patients with SSc-PAH, defined according to the Dana Point criteria. eGFR was calculated using the 4-variable Modified Diet in Renal Disease equation (MDRD); eGFR< 60 ml/min/1.73m2was considered abnormal. Demographic, serologic, physiologic, and hemodynamic parameters were compared between groups using t-tests or chi-squared analyses where appropriate.
Results: 133 SSc-PAH patients were included in this study (Table 1). Overall, 33% (44/133) of subjects had an eGFR<60 ml/min/1.73m2(low eGFR group). In general, the low eGFR group was older and was more likely to have diffuse disease, but there was no difference in gender, race, duration of SSc, or antibody profiles between groups. Pulmonary function and New York Heart Association functional class were similar between groups, however, six minute walk distance was significantly lower in the low eGFR group. Patients in the low eGFR group were more likely to have had a renal crisis and to have worse hemodynamics (higher mean pulmonary artery pressure, lower cardiac output, and higher pulmonary vascular resistance).
Table 1: Patient Population
Parameter |
Overall (n=133) |
eGFR<60 (n=44) |
eGFR>60 (n=89) |
p-value |
Age (years) |
60(11) |
64(9) |
59(11) |
<0.01 |
Gender (n,%women) |
109(83) |
38(86) |
71(82) |
0.49 |
Race (n,% Caucasian) |
120(90) |
42(95) |
78(88) |
0.15 |
Limited vs. Diffuse (n,% limited)* |
54(60) |
13(40) |
41(71) |
<0.01 |
Duration of SSc (yrs) |
8.2(9.5) |
7.7(9.5) |
8.9(9.4) |
0.68 |
First Raynaud’s symptom (yrs) |
14.3(12.0) |
14.9(13.3) |
13.9(11.4) |
0.69 |
History of renal crisis (n,%) |
6(5) |
5(13) |
1(1) |
<0.01 |
FVC (% predicted) |
81.7(15.7) |
84.9(15.4) |
79.9(15.7) |
0.10 |
FEV1/FVC (% predicted) |
81.7(9.9) |
82.0(11.1) |
80.7(9.9) |
0.51 |
TLC (%predicted) |
79.0(18.5) |
79.3(19.3) |
78.9(18.2) |
0.90 |
DLCO (%predicted) |
40.1(17.5) |
37.5(19.9) |
41.5(16.1) |
0.22 |
FVC/DLCO (% predicted) |
2.2(0.9) |
2.2(0.7) |
2.2(0.9) |
0.86 |
Home oxygen use (n,%) |
39(32) |
13(33) |
26(32) |
0.86 |
6MWT (m) |
332(127) |
291(134) |
355(119) |
0.02 |
NYHA class (I/II vs III/IV) |
62/57 |
20/23 |
42/34 |
0.52 |
Mean PAP (mmHg) |
37(19) |
40(11) |
36(9) |
0.02 |
CO (L/min) |
5.0(1.6) |
4.4(1.2) |
5.4(1.6) |
<0.001 |
PCWP (mmHg) |
10(3) |
9(3) |
11(3) |
0.03 |
PVR (Wood units) |
5.8(3.9) |
7.2(3.5) |
5.1(3.9) |
<0.01 |
Conclusion:
In this large cohort of patients with SSc-PAH, renal dysfunction was common and associated with more severe functional and hemodynamic impairments. Interestingly, there was no association between antibody profiles or race and prevalence of renal dysfunction. The determinants of renal disease and the relationship between renal dysfunction and mortality in this cohort will be the focus of future studies in the PHAROS registry.
Disclosure:
S. C. Mathai,
None;
L. K. Hummers,
None;
V. D. Steen,
Gilead,
5.
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