Vogt-Koyanagi-Harada disease presenting secondary to a post-infectious Mycoplasma pneumoniae autoimmune response.

PURPOSE: To report a rare case of Vogt-Koyanagi-Harada disease likely secondary to post-infectious Mycoplasma pneumoniae autoimmune response in a 14-year-old Hispanic female. OBSERVATIONS: On presentation, visual acuity was 20/400 in the right eye and 20/20 in the left eye. The patient also had bilateral hyperemia, subretinal fluid, and vitreous cell graded at 1+. Fluorescein angiography and indocyanine green chorioangiography showed bilateral peripapillary hypofluorescence consistent with blocking and hyperflourescence consistent with staining. Laboratory testing showed elevated M. pneumoniae IgM and rising IgG antibodies. Topical steroids and oral steroids helped mitigate the systemic disease process and fully restore visual acuity through the 7-week mark. CONCLUSIONS AND IMPORTANCE: The patient had elevated M. pneumoniae IgM and rising IgG antibodies resulting in ocular inflammation likely secondary to an autoimmune response. In this case of post-infectious M. pneumoniae, topical corticosteroids were beneficial in mitigating ocular manifestations initially, although oral steroids were needed and tapered over 6 weeks.

Achiasma and Kapur-Toriello syndrome: Two rare entities.

PURPOSE: Describe a unique case of achiasma in a patient with Kapur-Toriello syndrome. OBSERVATIONS: We provide a brief review of achiasma with common findings on examination and imaging studies, and a published classification system. In addition, consistent with the rare Kapur-Toriello syndrome, he had a right unilateral cleft lip and palate, neurologic abnormality in his achiasma, anal atresia, vesicoureteral reflux, hypospadias, and growth deficiency. CONCLUSIONS: and Importance: Achiasma is an incredibly rare neurological deficit and we present the first case of achiasma in Kapur-Toriello syndrome.

Effect of Tobacco Smoking on The Clinical, Histopathological, and Serological Manifestations of Sjögren's Syndrome.

OBJECTIVES: To assess the association of smoking habits with the clinical, serological, and histopathological manifestations of Sjögren's syndrome (SS) and non-Sjögren's sicca (non-SS sicca). METHODS: Cross-sectional case-control study of 1288 patients with sicca symptoms (587 SS and 701 non-SS sicca) evaluated in a multi-disciplinary research clinic. Smoking patterns were obtained from questionnaire data and disease-related clinical and laboratory data were compared between current, past, ever, and never smokers. RESULTS: Current smoking rates were 4.6% for SS patients compared to 14.1% in non-SS sicca (p = 5.17x10E-09), 18% in a local lupus cohort (p = 1.13x10E-14) and 16.8% in the community (p = 4.12x10E-15). Current smoking was protective against SS classification (OR 0.35, 95%CI 0.22-0.56, FDR q = 1.9E10-05), focal lymphocytic sialadenitis (OR 0.26, 95%CI 0.15-0.44, FDR q = 1.52x10E-06), focus score ≥1 (OR 0.22, 95%CI 0.13-0.39, FDR q = 1.43x10E-07), and anti-Ro/SSA(+) (OR 0.36, 95%CI 0.2-0.64, FDR q = 0.0009); ever smoking was protective against the same features and against anti-La/SSB(+) (OR 0.52, 95%CI 0.39-0.70, FDR q = 5.82x10E-05). Duration of smoking was inversely correlated with SS even after controlling for socioeconomic status, BMI, alcohol and caffeine consumption. CONCLUSIONS: Current tobacco smoking is negatively and independently associated with SS, protecting against disease-associated humoral and cellular autoimmunity. The overall smoking rate amongst SS patients is significantly lower than in matched populations and the effects of smoking are proportional to exposure duration. In spite of the protective effects of tobacco on SS manifestations, it is associated with other serious comorbidities such as lung disease, cardiovascular risk and malignancy, and should thus be strongly discouraged in patients with sicca.