Behçet uveitis: Current practice and future perspectives

Abstract

Described as early as Hippocrates in his “Third Book of Endemic Diseases,” Behçet's Disease (BD), also known as “The Silk Road Disease” following its initial demographics, consists of a triad of recurrent oro-genital ulcers and associated uveitis. Current demographics and rising percentages of patients seen far beyond the Silk Road in Ocular Inflammatory Disease and Uveitis Clinics list BD uveitis as one of the frontliners of non-infectious autoinflammatory eye diseases. Clinical features of BD and juvenile-onset BD are detailed alongside various approaches in classification and suggested algorithms for diagnosis that are outlined in this review. With the ongoing Human Microbiome Project and studies such as the MAMBA study, the role of the human microbiome in BD is highlighted in the pathophysiology of BD to include the current research and literature perspective. Furthermore, with the advancement of recent diagnostic and investigative techniques, especially in the field of Optical Coherence Tomography (OCT), disease-related characteristics are updated to encompass SD, EDI and OCT-angiography characteristics of BD. Having entered the era of biologic therapy, the role of various specific cytokine-blocking biologic drugs, such as TNF-α inhibitors (e.g., adalimumab, infliximab), interferon α-2a inhibitors, IL-6 and IL-1 inhibitors are presented and contrasted alongside the conventional immunosuppressant drugs and the classic old gold standard: corticosteroids (systemic or local). Finally, with the ongoing SARS-CoV-2 pandemic, it was not possible to conclude the review without reviewing the latest evidence-based literature reporting BD morbidity in this era, the observed pattern and treatment recommendations as well as those related to reported post-vaccine complications and emergence of BD.

Acute vulvar ulcer as a possible adverse event of gene‐basedCOVID‐19 vaccines: A review of 14 cases

“…The interval from vaccine receipt to ulcer onset was 2.4 ± 1.2 (0–4) days ( n = 14); This skewed distribution, with a sharp peak at 2 days after vaccine receipt, do not support coincidental association between vaccination and ulceration (Figure 2). The interval from the vaccine receipt to ulcer resolution was 16.9 ± 7.4 7–28 ( n = 10). In all 13 cases with a described prognosis, the ulcer resolved.…”

“…A 12-year-old girl who had never experienced sexual intercourse developed a fever of 40 C and vulvar sore a 26 and one SARS-COV2 PCRpositive vulvar ulcer case with a history of COVID-19 vaccination 4 months before ulcer onset were excluded. 27 There were 4 articles that were not the case reports: a review on Behçet's disease that referred to oro-genital ulcers, 28 an original article on a potential COVID drug, 29 and 2 editorials on vulvar ulcers following COVID-19 vaccination. 18,30 day after the second dose of the BNT162b2 (Pfizer) vaccine.…”

Decoding Behcet’s Uveitis: an In-depth review of pathogenesis and therapeutic advances

“…It is interesting to note that human heat shock proteins exhibit similarities to these epitopes, potentially leading to cross-reactive immune responses and subsequent autoimmune reactions [ 152 , 153 ]. In another study, specific streptococcal strains were isolated from BD patients with extraocular myopathy [ 154 ].…”

Commentary

“…A standalone positive typing for HLA-B51 is not considered diagnostic, and the diagnosis of this uveitic entity is based on a myriad of multisystem clinical signs that make up the diagnostic criteria. [ 1 ]…”

“…This recent understanding has expanded treatment horizons beyond standard treatment protocols, to include biologic therapy, especially tumor necrosis factor-alpha (TNF-α) antagonists, to achieve control of repeated disrupted immune response. [ 1 ] Progressive sight-threatening Behçet’s disease involving the entire uveal tract blinds up to 25% of patients within a course of 10 years, after which the disease progression tends to stabilize. [ 1 , 2 ]…”