Session Information
Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s - Clinical Aspects and Therapeutics II
Session Type: Abstract Submissions (ACR)
Background/Purpose:
Systemic Sclerosis (SSc) is associated with occlusive vasculopathy resulting in digital ischaemia, telangiectasia, scleroderma renal crisis (SRC) and pulmonary arterial hypertension (PAH). SRC and PAH have similar histological features with neointimal proliferation and medial hyperplasia and shared pathogenic mechanisms are plausible. Pulmonary hypertension (PH) after SRC is frequently observed but generally due to post capillary mechanisms with elevated post capillary wedge pressure (WHO Group II). We have explored the association of PAH (WHO Group I) with SRC based upon shared histopathological features and possible similarities in pathogenesis.
Methods:
Of more than 2000 SSc patients attending our institution between 1990-2013, 150 patients had a confirmed diagnosis of SRC of which six (4%) patients had a diagnosis of PAH (mean pulmonary arterial pressure ≥25mmHg and pulmonary capillary wedge pressure <15mmHg) following right heart catherisation. Estimated glomerular filtration rate (eGFR) was calculated using the 4-variable Modified Diet in Renal Disease (MDRD) equation. Demographic, clinical and haemodynamic parameters are expressed as mean ± SD.
Results:
Two patients had diffuse cutaneous SSc (dcSSc) and 4 patients had limited cutaneous SSc (lcSSc). 5/6 (83%) demonstrated an immunofluoresecence and ENA reactivity consistent with anti-RNA polymerase III reactivity, confirmed by specific ELISA. All patients with SRC and PAH were female. PAH was diagnosed after SRC in all patients (49 vs 44 years old). 4/6 (66.7%) patients required dialysis. 2/6 (33.3%) patients who did not require dialysis had an eGFR of 26 and 44mls/min/1.73m2. 2 patients recovered renal function at 6 and 14 months after the diagnosis of SRC. The mean mPAP (mean pulmonary arterial pressure) was 42 ± 12mmHg with a mean pulmonary capillary wedge pressure (PCWP) 9.3 ± 2.6mmHg and a cardiac index of 5.2 ± 0.8 L/min. 5 patients were treated with Bosentan and 1 patient with intravenous Iloprost. 3/6 (50%) patients died with a mean survival from date of diagnosis of SRC of 112 ± 30 months and a mean survival from date of diagnosis of PAH of 47 ± 17 months.
Conclusion:
The presence of SRC and PAH in the same patient is very rare and PAH occurred after SRC in all patients at a mean of 5 years later. Temporal separation of SRC and PAH suggests that these complications may occur independently, but the frequent association with ARA suggests common susceptibility factors may be relevant.
Disclosure:
B. Lynch,
None;
V. Sobanski,
None;
C. Handler,
None;
B. E. Schreiber,
None;
J. G. Coghlan,
None;
V. H. Ong,
None;
A. Burns,
None;
C. P. Denton,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/scleroderma-renal-crisis-and-pulmonary-arterial-hypertension-are-very-rare-in-the-same-patient/