Clinical Features of Syphilis Patients with Ocular Symptoms as the Initial Manifestation.

Purpose: This study aimed to investigate the clinical features of patients with syphilis having ocular symptoms as the initial manifestation. Patients and Methods: Eleven patients diagnosed with ocular syphilis at Dokkyo Medical University Hospital and Jichi Medical University Hospital between November 2018 and April 2022 were studied retrospectively. Results: Six patients were diagnosed with secondary, three were latent, and one was tertiary stage syphilis. Ten out of 11 patients underwent cerebrospinal fluid analysis, and 1 refused. Nine out of 10 patients tested positive, of which 4 presented with neurological symptoms and the others were asymptomatic. Nine out of 11 patients tested negative for human immunodeficiency virus. Antiluetic therapy was administered to 10 out of 11 patients, which improved or maintained visual acuity at -0.1 logMAR in 9 patients. One patient achieved the best-corrected visual acuity -0.1 logMAR in one eye, whereas the other showed no improvement due to severe chorioretinal degeneration. Conclusion: Ocular syphilis presents with various clinical findings and has no significant ocular manifestations without acute syphilitic posterior placoid chorioretinitis. Patients diagnosed with syphilis based on ocular symptoms should undergo cerebrospinal fluid analysis.

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.

Clinical characteristics of scleritis patients with emphasized comparison of associated systemic diseases (anti-neutrophil cytoplasmic antibody-associated vasculitis and rheumatoid arthritis).

PURPOSE: We evaluated patient profiles, clinical features, associated systemic diseases, treatment modalities, and ocular complications in cases of scleritis and episcleritis. STUDY DESIGN: Retrospective. METHODS: Clinical data of 128 patients referred to the ophthalmology clinic at Jichi Medical University Hospital during the 4-year period from April 2011 to March 2015, and diagnosed with scleritis or episcleritis were examined. Gender, average onset age, unilateral or bilateral manifestation, classification type, associated systemic diseases, and treatments were retrospectively investigated. RESULTS: The cohort consisted of 57 men and 71 women. Average onset age was 54.3 ± 17.4 years. Diffuse anterior scleritis was the most common type. It was noted in 43 (32.8%) patients, followed by episcleritis in 35 (27.3%), nodular anterior scleritis in 23 (18.0%), necrotizing anterior scleritis in 22 (17.2%), and posterior scleritis in 6 (4.7%). Eighteen (81.8%) of 22 patients with necrotizing anterior scleritis required some type of systemic medication, including corticosteroid, cyclophosphamide, cyclosporine, azathioprine, methotrexate, or rituximab administration. Forty (31.3%) had associated systemic diseases, which included 10 with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and 8 with rheumatoid arthritis (RA). Patients with ANCA-associated vasculitis had a tendency to develop scleritis first and had significantly worse visual prognoses compared to those with RA. CONCLUSIONS: Approximately 30% of the patients with scleritis and episcleritis had complications involving systemic diseases, including ten patients with ANCA-associated vasculitis and 8 with RA. ANCA-associated vasculitis was more often diagnosed after scleritis and patients suffered poorer visual prognoses than those with RA.

An unusual presentation of concurrent congenital grouped pigmentation of the retina with albinotic spots.

A 10-year-old Japanese girl presented with multiple, small, flat, dark brown clusters of retinal pigment epithelium in both eyes. She had no other symptoms. The appearance was consistent with congenital grouped pigmentation of the retina or bear track spots. A unique co-existing feature was the presence of small, albinotic spots within the entire retina. Fluorescein angiography demonstrated a persistent hypofluorescence correlating with the grouped pigmentation of the retina and granular hyperfluorescence correlating with the albinotic spots. Her mother also showed irregular pigmentation and albinotic spots in both eyes. We describe an extremely rare case of congenital grouped pigmentation and albinotic spots occurring in the retina of the same individual.

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.

Persistent presence of the anti-myelin oligodendrocyte glycoprotein autoantibody in a pediatric case of acute disseminated encephalomyelitis followed by optic neuritis.

We report the case of a 5-year-old Japanese girl who initially had acute disseminated encephalomyelitis (ADEM) and was positive for the myelin oligodendrocyte glycoprotein (MOG) antibodies and developed unilateral optic neuritis (ON) 71 days after ADEM onset. The patient's serum was positive for the anti-MOG antibodies from the onset of ADEM to the development of ON. This phenotype has been reported in only two previous articles, and the specific mechanism of action of the anti-MOG antibodies is not yet understood. Our case suggests that the anti-MOG antibody can be associated with the pathogenesis of ADEM followed by ON. Thus, patients with ADEM who test positive for the anti-MOG antibody may be at risk of developing subsequent ON.