-S 392 -
■ S-659 ■
A case of sensori-motor polyneuropathy in a patient with Sjögren’s syndrome
조선대학교병원
*김은정, 김윤성, 김현숙
Sjögren’s syndrome is a chronic inflammatory disorder characterized by lymphocytic infiltration of exocrine glands, especially the lacrimal and salivary glands. Although it is primarily characterized by a particular form of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia), this condition may affect a wide variety of organ systems including skin, lung, heart, kidney and rarely neurological manifestations. Peripheral neuropathy results from nerve damage due to vasculitis or demyelinating of nerve sheath, and becomes to damage the ability to sending messages. We reports the case of a 71-year-old woman who came to the hospital due to a polyneuropathy of lower limb and was diagnosed as having Sjögren’s syndrome but progressed to the muscle atrophy during the treatment.
■ S-660 ■
Pulmonary Alveolar Proteinosis as an Unusual Pattern of Lung Involvement in Sjögren Syndrome
Divisions of 1Rheumatology and 2Pulmonology, Department of Internal Medicine, Konkuk University School of Medicine, Seoul, Korea
*Eugene Park
1, Hee-Jung Kim
2, So-Mi Kim
1, Choi Lee
1, Mi-jin Hong
1, Jang-Soo Han
1, Jung-Hee Park
1, Sung-Chul Hong
1, Sang-Heon Lee
1, Hae-Rim Kim
1Pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates within alveoli. Secondary PAP has been reported in association with various diseases including hematological disorders, immunological diseases, lysinuric protein intolerance, infection and medications. PAP is related to the presence of anti-GM-CSF Ab, and therefore it is thought as a kind of autoimmune disorder.
Here, we describe a case of PAP in patient with primary Sjögren syndrome (SS). A 45-year-old nonsmoking woman was hospitalized due to dyspnea and dry cough for 2 months. She did not have fever, chill, or sputum. For several years, she felt dry eyes and mouth and she had used artificial tears. Expiratory crackles were heard in both lower lobes and clubbed fingers were observed. In the laboratory studies, WBC 10,920/mm3 (neutrophil 65.6%), hemoglobin 15.5 g/dL, hematocrit 44.1%, ESR 43 mm/h, CRP was 0.05 mg/dL. ANA was 1:80 with speckled pattern, and anti-SSA and anti-SSB antibodies were positive in line immunoassay method. Computed tomography scans showed bilateral alveolar infiltration with diffuse ground glass opacity. Fluid from bronchoalveolar lavage (BAL) was opaque and milky colored. There were many PAS positive foamy macrophages in the BAL fluid. The histology of lung showed that alveolar spaces contained histiocytes and eosinophilic proteinous materials with positive surfactant immunostaining. Salivary gland scan showed markedly decreased uptakes in parotid and mandibular glands. On the basis of imaging and histological findings, she was diagnosed as having PAP with primary SS. Whole lung lavages of both lungs were performed but her symptoms and imaging results were not improved. Then, monthly intravenous cyslophosphamide was administered, but the PAP was not improved.