Background/Purpose:  A few isolated case reports of precapillary pulmonary hypertension (PH) in ANCA-associated vasculitides, mostly granulomatosis with polyangiitis (GPA), have been published. The characteristics and outcomes of PH–GPA patients entered in the FVSG database were analyzed.

Methods:  GPA patients with precapillary PH were included from this series. GPA diagnosis was based on ACR classification criteria or the revised Chapel Hill Conference Consensus (CHCC) nomenclature. Patients with connective tissue disease or undergoing hemodialysis were excluded. Chronic thromboembolic pulmonary hypertension required appropriate investigations to be excluded. Precapillary PH was defined as mean pulmonary arterial pressure (mPAP) ≥25 mm Hg at rest, with mean pulmonary capillary wedge pressure (PCWP) ≤15 mm Hg, measured during right heart catheterization (RHC).

Results:  Among the 1065 GPA patients entered in the database, 3 male (0.3%) had precapillary PH; mean age, 43.1 years. All had systemic GPA according to the revised CHCC nomenclature and ACR criteria. Precapillary PH was diagnosed 4 years before GPA in 1 patient and after an average of 1.8 years for the others. All patients had NYHA III dyspnea and confirmed precapillary PH at RHC. In 1 patient, lung biopsy showed histological evidence of granulomatous vasculitis, and arteriolar and venous involvement; a ventilation–perfusion scan detected multiple perfusion defects consistent with thromboembolic disease that was ruled out by pulmonary angiography showing poor peripheral perfusion with a typical “dead tree” aspect, likely due to small-vessel vasculitis. At PH diagnosis, in combination with high-dose glucocorticoids, cyclophosphamide or rituximab was continued in 1 patient each. PH treatment was prescribed only in the patient with preexisting PH who received continuous epoprostenol infusions. Repeat RHC showed significant clinical and hemodynamic improvement in the 3 patients; their mean mPAP decreased from 51.6 (range, 41–70) to 33.0 mm Hg (range, 29–42). The patient with preexisting PH died of sepsis during GPA induction therapy. After treatment, in the patient with multiple ventilation–perfusion-scan defects, no change was observed. With median follow-up post-PH diagnosis of 4.2 years, the 2 other patients are alive, with NYHA class II dyspnea and in complete GPA remission, with median daily prednisone dose at 2.5 mg.

Conclusion:  The data from this first series indicated that GPA can be a rare cause of precapillary PH. Lung small-vessel vasculitis is likely to be causative. GPA-caused precapillary PH may respond to combined high dose glucocorticoids and cyclophosphamide or rituximab.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/precapillary-pulmonary-hypertension-in-granulomatosis-with-polyangiitis-a-case-series-with-long-term-follow-up/