the initial laboratory evaluation revealed an erythrocyte sedimentation rate (ESR) of 61 mm/h (normal value <10) and a serum C-reactive protein (CRP) level of 12 mg/L (normal value <5). Rheumatoid factor was 184 uI/mL (normal range <20), and serum angiotensin-converting enzyme (SaCE) was 65 u/I (normal value 8-52 u/L). Full blood count and blood chemistry were all within the normal range. Immunoglobulin G levels were 21.3 g/L (normal values 7.0-16.0 g/L); kappa light chain 18.58 g/L (6.66-14.65 g/L); and lambda light chain 7.43 g/L (2.99-6.99 g/L), with no detection of monoclonal component. Purified protein derivative test result was negative (an induration <5 mm).her chest radiograph (Fig. 1a) and high-resolution computed tomography (hRCt) (Fig. 2a) showed hilar and mediastinal lymphadenopathy and multiple small pulmonary nodules. Lung function tests and carbon monoxide diffusing capacity were normal. Bronchoalveolar lavage demonstrated an increase in CD4+/CD8+ t cell ratio (12.4). mycobacterial and fungal cultures were negative. to investigate the pulmonary nodules, a mediastinoscopydirected biopsy was performed. the histological exam revealed multiple noncaseating granulomas.the diagnosis of sarcoidosis (stage II) was established based on the clinical, imaging, and pathological findings. the patient was diagnosed with coexisting pSS and sarcoidosis.Because the respiratory symptoms and fatigue worsened over time, the patient was empirically treated with prednisolone 0.3 mg/kg/day for 6 weeks, with a slow tapering over 6 months, associated with calcium supplementation and alendronic acid/colecalciferol (70 mg/5,600 uI, weekly) [1].Gradually, all the symptoms improved, and 1 year later, the patient denied any respiratory or systemic symptoms.Abstract herein, we describe a 44-year-old female diagnosed with histologically proven coexistence of primary Sjögren's syndrome and sarcoidosis with pulmonary and muscular involvement. the differential diagnosis may be difficult, but this is not an exceptional case, which highlights the need to critically revise the consideration of sarcoidosis as an exclusion for primary Sjögren's syndrome, as established in current classification criteria.Keywords Primary Sjögren's syndrome · Sarcoidosis · Lung · muscle
Case reporta 44-year-old Caucasian female was referred to our Department due to a 2-week history of dyspnea, persistent dry cough, arthralgia, and fatigue. Four years previously, primary Sjögren syndrome (pSS) had been diagnosed on the presence of sicca symptoms associated with SSa and SSB auto-antibodies, and a salivary gland biopsy demonstrating lymphocytic infiltrates (Chisholm-mason classification grade IV). She had been successfully treated with hydroxychloroquine 400 mg/day and an ophthalmic emulsion. her family and occupational and environmental histories were not contributory.Physical examination showed normal vital signs, and cardiovascular, pulmonary, and musculoskeletal clinical examinations were unremarkable. no peripheral adenopathies were noted.