Background Primary Sjögren Syndrome (pSS) has an estimated prevalence of 0.2%. Connective tissue disorders are known risk factors for causing pulmonary arterial hypertension (PAH). The association of PAH and pSS is rare.

Objectives To examine the incidence of PAH in pSS and to review the clinical, hemodynamic parameters and medical management of each case.

Methods We included 1,036 patients followed in a specialist Pulmonary Hypertension Centre between 1996-2010. History of appetite suppressant intake, thyroid hormone level and human immunodeficiency virus (HIV) serology were collected to rule out other causes of PAH. Cardiac echo-doppler excluded congenital heart disease. A ventilation/perfusion lung scan, a spiral computed tomography of the chest, and a pulmonary angiography if necessary were performed to exclude chronic thromboembolic pulmonary hypertension. Of 1036 patients, 79% had systemic sclerosis, 7% had SLE, 5% had mixed connective tissue disease, 2.5% had undifferentiated connective tissue disease, 2% had myositis and the remainder had overlap diseases. Seven patients (<1%) fulfilled the 2002 revised criteria for pSS proposed by the American-European Concensus group. All patients with secondary SS were excluded. In 7 patients with pSS who underwent a right heart catherisation (RHC), 4 patients had PAH (mean pulmonary arterial pressure ≥25mmHg and pulmonary capillary wedge pressure <15mmHg), 1 patient had post capillary pulmonary hypertension (mean pulmonary arterial pressure ≥25mmHg and pulmonary capillary wedge pressure >15mmHg) and 2 patients did not have pulmonary hypertension.

Results All patients with pSS and PAH were female with a mean age at PAH diagnosis of 61.8 ± 10.8 years. Clinical presentation of PAH was severe in all cases: WHO functional class (FC) was III in patients 1-3 and IV in patient 4. Mean 6-minute walk distance was 310 ± 105m (range, 152-448m). Overall, PAH was severe with a mean mPAP of 51 ± 12mmHg. Among the 4 patients, patients 1 and 2 received Bosentan therapy alone, patient 3 received a combination of Bosentan and Sildenafil and patient 4 received inhaled Iloprost alone. Patient 4 did not have a repeat RHC at 3-4 months. The remaining three patients showed a reduction in mPAP with no change in WHO FC at 3-4 months (Table 1). Two patients died from PAH at 35 (patient 2) and 26 (patient 4) months after diagnosis.

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Conclusions PAH in pSS is rare and to our knowledge, there are less than 50 published case reports in the English literature. We report a case series of four patients with pSS and PAH over 14 years in a specialist pulmonary hypertension centre. Standard PAH therapies (endothelin receptor antagonists, phosphodiesterase type 5 inhibitors or prostanoids) were initially effective but the best treatment strategy remains to be defined.

Disclosure of Interest None Declared