Atypical Sjögrenʼs Syndrome Initially Presenting as Lymphocytic Interstitial Pneumonitis followed by Immune Thrombocytopenia

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1. Background

Sjögren’s syndrome is a chronic systemic autoimmune disorder commonly characterized by dry eyes and dry mouth. It has an incidence rate of 7/100,000 persons annually [1]. In addition to the anti-SSA antibody, the oral and ocular symptoms are assessed in the most recent classification criteria for primary Sjögren’s syndrome [2]. However, extraglandular features may occur in a third of the cases and sometimes they are the presenting signs. These can include Raynaud phenomenon or cutaneous vasculitis, arthralgia or myopathy, interstitial lung disease (ILD), primary biliary cholangitis, interstitial nephritis, peripheral neuropathy, or lymphoma [3]. ILD is seen in only 9–20% cases of Sjögren’s syndrome, and of the types of ILD, lymphocytic interstitial pneumonitis (LIP) is less frequently identified [4]. LIP was first described in 1966 [5]. As opposed to pulmonary manifestations in Sjögren’s syndrome, immune thrombocytopenia (ITP) is not as well-recognized a feature. To the best of our knowledge, we know of no case report of LIP and ITP in Sjögren’s syndrome. We present one such case of Sjögren’s syndrome that presented as LIP and then developed ITP.

2. Case Report

A 74-year-old Ecuadorian man who smoked 3 cigarettes a day for a year but quit 15 years prior presented to the Emergency Department (ED) with four days of a productive cough that did not improve with azithromycin. His medical history included hypertension and cataracts. He worked at a clothing factory for years. The body mass index was 24.9 kg/m². Physical examination showed a blood pressure of 154/86 mmHg, pulse of 115 beats/minute, respiration rate of 18/min, oxygen saturation of 95% on ambient air, and temperature of 101°F. Pulmonary auscultation on hospital admission revealed decreased bilateral breath sounds in the lower lung fields. A chest radiograph showed a large rounded opacity in the superior segment right lower lobe and bibasilar patchy infiltrate. The results of laboratory studies revealed a white blood cell count of 16,000/μl with 82.7% neutrophils and 8% lymphocytes, hemoglobin of 11.9 g/dl, and platelet count of 250,000/μl. Blood and urine cultures yielded no growth, and his acute symptoms...