Impact of HLA-B51 on Uveitis and Retinal Vasculitis: Data from the AIDA International Network Registries on Ocular Inflammatory Disorders.

PURPOSE: The clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Behçet's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown. METHODS: Data were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time. RESULTS: Records of 213 patients (341 eyes) were analyzed. No differences in complications were observed (p = 0.465). With regard to VA, a significant difference was detected in median BCVA (p = 0.046), which was not maintained after Bonferroni correction (p = 0.060). RV was significantly more prevalent in NIU-affected patients who tested positive for HLA-B51, irrespective of the systemic diagnosis of BD (p = 0.025). No differences emerged in the occurrence of macular edema (p = 0.99). CONCLUSIONS: Patients with NIU testing positive for HLA-B51 exhibit an increased likelihood of RV throughout disease course, irrespective of a systemic diagnosis of BD. The rate of complications as well as VA are comparable between NIU cases unrelated to BD testing positive for HLA-B51 and uveitis associated with BD. Therefore, it is advisable to perform the HLA-B typing in patients with NIU or retinal vasculitis, even in the absence of typical BD features.

Neurosarcoidosis-Induced Hypophysitis Mimicking Pituitary Macroadenoma.

Sarcoidosis is characterized by the presence of noncaseating granulomatous inflammation in the affected organs. Isolated involvement of the hypothalamic-pituitary axis in patients with sarcoidosis is rare. We report a rare case of a female patient in whom hypophysitis, mimicking a pituitary macroadenoma, resulted in pituitary transsphenoidal surgery. A female patient had been complaining of bilateral temporal headaches for over a month. Brain MRI showed a pituitary adenoma of height 16 mm, width 16 mm, and depth 12 mm. Hormonal assay showed central hypothyroidism and elevated level of prolactin. Histological examination revealed granulomatous hypophysitis. A specific search for Mycobacterium tuberculosis was negative on the pituitary tissue. After the exclusion of differential diagnoses, the combination of clinical, laboratory and radiological tests led to the diagnosis of neurosarcoidosis. This report presents an uncommon case of a pituitary localization of neurosarcoidosis mimicking a macroadenoma. Understanding the different MRI aspects of neurosarcoidosis is essential to avoid interpretive blunders that could result in an incorrect diagnosis.

Rituximab-induced mild encephalopathy with a reversible splenial lesion syndrome (MERS): An adverse effect to add to the list.

Mild encephalopathy with a reversible splenial lesion syndrome (MERS) is a rare clinico-radiological entity. Rituximab (RTX)-induced MERS has never been described before. Herein, we report the case of a 33-year-old patient diagnosed since 2017, with an IgG4-related disease (IgG4-RD). This diagnosis was retained in the face of a prolonged fever, sicca syndrome, hepatic damage and renal pseudotumour associated to a high level of IGg4 at 2.8 g/L with suggestive renal histology. The patient was treated with corticosteroid therapy with persistence of renal impairment and nephrotic syndrome indicating RTX therapy. The patient received his first dose of RTX and presented neurological and respiratory impairments a few hours afterwards. An infectious investigation comprising a SARS CoV-2 PCR and viral PCRs (VZV, herpes and CMV) on cerebrospinal fluid (CSF) were negative. The HBV, HCV, HIV, Parvo B19, CMV, EBV, herpes, mycoplasma and syphilis serologies as well as Legionella antigenuria were also negative. The patient had received intravenous immunoglobulins (IVIG) and methylprednisone, associated with sodium valproate with favourable outcome. The diagnosis of MERS induced by RTX was retained in our patient according to clinical and radiological features. We herein report the first case of MERS following RTX in a patient treated for IgG4-RD.

[Drug-induced vasculitis]. / Vascularites médicamenteuses : à propos d'une série de 13 cas.

INTRODUCTION: Drug-induced vasculitis is reported in almost 10-20 % of vasculitis. Several drugs may be incriminated in their occurrence. Our study aimed to study the epidemiological, clinical, histopathological and evolutionary characteristics of drug-indced vasculitis from a series of cases and to specify the different drugs involved. METHODS: We conducted a retrospective study during the period from January 2006 to December 2015 from the cases notified to the regional pharmacovigilance center of Sousse, Tunisia. The diagnosis was established according to the criteria proposed by the group of the American college of rheumatology (ACR). RESULTS: Our study included thirteen cases of drug-induced vasculitis over a ten-year period, with an mean incidence of 1.3 new cases per year. Mean age of patients was 40.84 years. The mean delay from the treatment onset was 14.46 days with extremes ranging from 5 days to six weeks. Most patients had pure skin involvement. Association with other extracutaneous complaints was present in five cases. Cutaneous biopsy was performed in all patients showing a pathological pattern of leukocytoclastic vasculitis, associated with fibrinoid necrosis, extravasation of red blood cells and allergic capillaritis. The outcome was favorable for all patients. The offending drugs in our series were amoxicillin, pristinamycin, rifampicin, fluconazole, metformin, glimepiride, phenobarbital, gabapentin, fenofibrate, ibuprofen, allopurinol, rituximab and tinzaparin. CONCLUSION: Anamnestic, clinical, biological and histopathological findings allow the early recognition of drug-induced vasculitis. Adequate treatment prevents systemic spreading and a worse prognosis.

Bloom syndrome complicated by colonic cancer in a young Tunisian woman.

Bloom syndrome (BS) is an autosomal recessive inherited disorder characterized by chromosomal instability leading to a high risk of cancer at an early age. The diagnosis should be considered in patients with short stature, photosensitivity, variable degrees of immunodeficiency, and hypogonadism. We report a 19-year-old woman, with history of dysmorphic features and recurrent infections. The diagnosis of bloom syndrome was made and confirmed cytogenetically at the age of 14 years. She developed a colon cancer revealed by venous thrombosis and anemia. She died after 15 days of the cancer diagnosis. This is the first registrated case of confirmed Bloom syndrome in Tunisian population.