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Pulmonary arterial hypertension as initial manifestation of primary Sjögren's syndrome

Jairia Dela Cruz
04 May 2018

Pulmonary arterial hypertension (PAH) may be the first manifestation of primary Sjögren's syndrome (pSS) in patients with Raynaud’s phenomenon, high-titre rheumatoid factor, hepatic injury or pericardial effusion, according to a recent study.

The case–control study included 29 patients with pSS–PAH (mean age at enrolment, 40.3 years; mean duration of PAH, 24.7 months) and 116 pSS controls without PAH (mean age at enrolment, 40.1 years). In the pSS-PAH group, 12 patients (41.4 percent) presented with PAH as the initial manifestation of the autoimmune disease, and shortness of breath was most common symptom (29/29; 100 percent). [Int J Rheum Dis 2018;doi:10.1111/1756-185X.13290]

Patients with pSS-PAH had a mean pulmonary arterial pressure of 51.8 mm Hg, mean cardiac index of 2.3 L/min/m2 and mean pulmonary vascular resistance of 13.0 Wood units. Majority received immunosuppressive agents (93.1 percent) and PAH‐targeted therapies (86.2 percent).

On univariate binary logistic regression analysis, four independent risk factors for PAH in pSS emerged, namely Raynaud's phenomenon (odds ratio [OR], 9.660; p=0.000), rheumatoid factor ≥200 U/mL (OR, 6.691; p=0.001), hepatic injury (OR, 3.284; p=0.008) and pericardial effusion (OR, 3.279; p=0.016).

“In summary, our results indicate that pSS patients with Raynaud’s phenomenon, high-titre rheumatoid factor, hepatic injury or pericardial effusion should be screened for PAH for early diagnosis and treatment,” the investigators said.

They also pointed out that symptoms of PAH are easily overlooked, and shortness of breath—the most common symptom of PAH in pSS patients—is not specific and can be explained by other causes such as interstitial lung disease.

The study was carried out at a tertiary hospital and might be limited by the possibility that some patients had been treated at local hospitals prior to PAH diagnosis.

Additional work is necessary to validate the current findings, as well as to evaluate the efficacy of steroid and immunosuppressive therapy in patients with pSS–PAH, the investigators said.

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