Characteristics of 68 patients with clinically proven sarcoidosis based on the Japan Society of Sarcoidosis and Other Granulomatous Disorders 2015 criteria.

BACKGROUND: The presence of histologically evident epithelioid granuloma is required for the diagnosis of sarcoidosis worldwide. The Japan Society of Sarcoidosis and Other Granulomatous Disorders 2015 diagnostic criteria (JSSOG 2015 criteria) includes "clinically proven diagnosis" (involvement of at least 2 of 3 systems confirmed solely by clinical assessment) because of the frequency of sarcoidosis with ocular, cardiac, and respiratory involvement in Japan and the difficulty of obtaining specimens. Here, we describe in detail the clinical presentation of clinically diagnosed sarcoidosis. METHODS: We enrolled 68 consecutive patients with clinically diagnosed sarcoidosis (12 men, 56 women) based on the JSSOG 2015 criteria who were treated at Jichi Medical University between December 2018 and January 2000. We analyzed age at diagnosis, organ involvement, and laboratory findings. RESULTS: Age at diagnosis was unimodal in women. Ocular, splenic, cardiac, and skin involvement, and hypercalcemia were observed in 95.6%, 8.8%, 7.4%, 5.9%, and 35.0% of patients, respectively. High serum lysozyme and soluble interleukin-2 receptor (sIL-2R) levels, bilateral hilar lymphadenopathy on chest radiography, high-grade atrioventricular block or fatal ventricular arrhythmia, and bundle branch block were found in 18.8%, 48.3%, 95.6%, 5.0%, and 10.0% of patients, respectively. CONCLUSIONS: The age-specific distribution of clinically diagnosed sarcoidosis was similar to histologically diagnosed sarcoidosis in women, as previously reported. Rates of elevated serum lysozyme and sIL-2R levels were lower in this study than previously reported in histologically diagnosed patients in Japan.

Central retinal vein occlusion caused by hyperviscosity syndrome in a young patient with Sjögren's syndrome and MALT lymphoma.

We report in this article central retinal vein occlusion (CRVO) caused by hyperviscosity syndrome (HVS) in a young patient with mucosa-associated lymphoid tissue (MALT) lymphoma and Sjögren's syndrome (SjS). A 32-year-old female was referred to our hospital from a local ophthalmologist. Fundoscopic examination and fluorescein angiogram revealed she had a serous retinal detachment in the right eye, together with CRVO (nonischemic type) in both eyes. Systemic examinations revealed hyperglobulinemia, increased blood viscosity, increased antinuclear antibody, increased rheumatoid arthritis particle aggregation, and increased anti-SS-A antibody. Together with a decreased salivary gland secretory function, she was eventually diagnosed as suffering from SjS. Moreover, a large cystic mass was found in the anterior mediastinum on the chest X-ray. Fine needle biopsy soon revealed she had MALT lymphoma. After eight courses of the administration of rituximab, cyclophosphamide, vincristine, and prednisolone (R-CVP), most laboratory values were normalized, including blood viscosity. Cystic mass in the anterior mediastinum decreased, and the conditions of CRVO in both eyes had much improved. Decreased best-corrected visual acuity (BCVA) in the right eye was fully restored by sixth month. Not only MALT lymphoma, but also SjS can cause secondary hyperglobulinemia. Indeed, immunoelectrophoresis-serum test showed a polyclonal pattern of hyperglobulinemia. Therefore, SjS was thought to be the primary reason of hyperglobulinemia in this patient, which induced HVS, eventually causing CRVO. R-CVP therapy was effective for not only MALT lymphoma but also SjS accompanied with HVS. Consequently, R-CVP therapy led to the improvement of CRVO.