Report Shows Rituximab May Help Treat MCTD-Associated PAH

Compared with idiopathic pulmonary arterial hypertension, PAH associated with CTD occurs predominantly in older women and features progressive symptoms (e.g., dyspnea, chest pain, palpitations), leading to a shorter survival.²

The pathogenesis of PAH associated with CTD isn’t clearly understood. A study done by Quismorio et al proposed that the deposition of immune complexes within the pulmonary vascular endothelium results in dysfunction leading to PAH.³ Other investigators propose that PAH may develop secondary to direct proliferative vascular involvement associated with interstitial fibrosis and pulmonary venous hypertension due to left-side heart disease.⁴

Due to this theory, the use of immunosuppressants and steroids may prove more suitable for treatment compared with directed therapies with endothelin receptor antagonists and phosphodiesterase-5 inhibitors.^4,5 Rituximab delivers significant immunomodulatory effects in autoimmune and hematologic pathologies. As our case suggests, rituximab may show promise as a primary therapy in the treatment of PAH-associated with CTD.

Josna Haritha, MD, MPH, completed medical school at the University of Alabama in 2013 and finished an internal medicine residency at Virginia Commonwealth University (VCU) in 2016. She is a first-year rheumatology fellow at the University of Chicago.

Huzaefah Syed, MD, joined the VCU faculty in 2012 as a rheumatology clinician-educator at the level of assistant professor. She completed all of her training at VCU School of Medicine and has a special interest in rheumatoid arthritis and sarcoidosis.

Abhishek Nandan, MD, completed medical school at Michigan State University, finished an internal medicine residency at VCU in 2016 and is currently a second-year rheumatology fellow at VCU.

Daniel Grinnan, MD, is an associate professor in pulmonary and critical care at VCU and director of VCU’s comprehensive care center for pulmonary hypertension.

References

1. Chung, SM, Lee CK, Lee EY, et al. Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension. Clin Rheumatol. 2006 Nov;25(6):866–872.
2. Galiè N, Manes A, Farahani KV, et al. Pulmonary arterial hypertension associated to connective tissue disease. Lupus. 2005;14(9):713–717.
3. Quismorio FP Jr., Sharma O, Koss M, et al. Immunopathologic and clinical studies in pulmonary hypertension associated with systemic lupus erythematosus. Semin Arthritis Rheum. 1984 May;13(4):349–359.
4. Denton CP, Pope JE, Peter HH, et al. Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Ann Rheum Dis. 2008 Sep;67(9):1222–1228.
5. Galiè N, Olschewski H, Oudiz RJ, et al. Ambrisentan for the treatment of pulmonary arterial hypertension: Results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation. 2008 June 10;117(23):3010–3019.