Sjogren’s syndrome is a chronic inflammatory condition primarily affecting exocrine glands namely lacrimal and salivary. Extraglandular manifestations occur in approximately 25 percent of patients. Pulmonary involvement is most frequently characterized by a persistent dry cough as well as non-allergic rhinitis and sinusitis. Cases of small airway disease, lymphocytic interstitial pneumonitis (LIP), and interstitial fibrosis have been reported.
A 48 year old women Sjogren’s associated lymphocytic interstitial pneumonitis which had been stable on hydroxycholoquine presented with worsening dyspnea on exertion. Her examination was significant for jugular venous distention, left parasternal lift, hepatojugular reflux and bilateral 3 + lower extremity edema. A right heart catheterization confirmed pulmonary arterial hypertension (PAH) with PA pressures of 95/39 mm Hg mean pulmonary artery pressure of 62 mmHg and a pulmonary artery occlusion pressure of 9 mmHg. The cardiac output was 8 L/min with a cardiac index of 3.8 L/min/sq meter. A ventilation-perfusion scan was low probability. Her PAH was initially treated with a regimen consisting of coumadin, furosemide and sildenafil. There was significant symptomatic improvement however 6 months later she complained of recurrent fevers and decompensated right ventricular failure requiring initiation of inhaled iloprost. Further evaluation confirmed low grade lymphoma she was treated with a regimen of etoposide, vincristine, doxarubacin, cyclophosphamide and oral prednisone. She achieved complete remission after one year of therapy.
Sjogren’s syndrome is a common autoimmune process which oftentimes has pulmonary involvement however pulmonary arterial hypertension is rare. PAH frequently complicates scleroderma, however only 32 cases have been reported in the literature of PAH in the setting of Sjogren’s syndrome. Our patient was unique in that she developed several pulmonary complications of Sjogren’s syndrome. She had LIP, which is well recognized in Sjogren’s syndrome, PAH which is rare, and then developed lymphoma. Each of these complications has been successfully treated and she is now NYHA functional class II on selective vasodilator therapy.
Pulmonary complications in Sjogren’s syndrome are common. LIP is the most common pulmonary complication and may convert to lymphoma at any point in the disease course. However PAH may also occur in a small percentage of cases and responds well to selective vasodilator therapy.
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