Frosted Branch Angiitis Secondary to Familial Mediterranean Fever Resembling Central Retinal Vein Occlusion

Özateş, Serdar; Özdal, Pınar; Teke, Mehmet Yasin

doi:10.1155/2016/2916027

Cited by 8 publications

(10 citation statements)

References 10 publications

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“…Unclassified vasculitis was described in a total of 11 FMF patients from various case reports (74–80, 84–87). FMF was associated with unclassified medium vessel vasculitis in three patients (patient 1, 2, 3).…”

Section: Resultsmentioning

confidence: 99%

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Association of Vasculitis and Familial Mediterranean Fever

et al. 2019

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Certain types of vasculitis occur more frequently and present differently in patients with familial Mediterranean fever (FMF). We assessed the characteristics of patients with FMF and systemic vasculitis through a systematic review of the literature. Medline was searched by two independent investigators until December 2017. We screened 310 articles and selected 58 of them (IgA vasculitis n = 12, polyarteritis nodosa (PAN) n = 25, Behçet's disease (BD) n = 7, other vasculitis n = 14). Clinical case reports were available for 167 patients (IgA vasculitis n = 46, PAN n = 61, BD n = 46, other vasculitis n = 14), and unavailable for 45 patients (IgA vasculitis n = 38, PAN n = 7). IgA vasculitis was the most common vasculitis in FMF patients with a prevalence of 2.7–7%, followed by PAN with a prevalence of 0.9–1.4%. Characteristics of FMF did not differ between patients with and without vasculitis. Patients with FMF and IgA vasculitis displayed more intussusception (8.7%) and possibly less IgA deposits on histological analysis than patients with IgA vasculitis alone. Patients with FMF and PAN had a younger age at vasculitis onset (mean age = 17.9 years), as well as more perirenal hematomas (49%) and CNS involvement (31%) than patients with PAN alone. Glomerular involvement was noted in 33% of patients diagnosed with PAN, suggesting an alternative diagnosis. Sequencing of the MEFV gene confirmed the presence of two pathogenic variants in 73% of FMF patients with IgA vasculitis or PAN. The majority of patients with BD were from one case series, and presented more skin, gastrointestinal, and CNS involvement than patients with isolated BD. In conclusion, FMF, particularly when supported by two pathogenic MEFV mutations, could predispose to IgA vasculitis, or a PAN-like vasculitis with more perirenal bleeding and CNS involvement.

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Section: Resultsmentioning

confidence: 99%

“…The co-occurrence of FMF and unclassified small vessel vasculitis was described in seven patients (patients 4, 5, 6, 7, 12, 13, and 14) (77–80, 85–87). Vasculitis phenotypes were heterogeneous.…”

Section: Resultsmentioning

confidence: 99%

Association of Vasculitis and Familial Mediterranean Fever

et al. 2019

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“…Substantial overlap between pathogenic mechanisms of both diseases seems possible. [11,13,14,28]. However, although the role of several in ammasomes has been discovered in the pathogenesis of several OIDs, the pyrin in ammasome has not been shown to be involved in any OIDs to date [29].…”

Section: Discussionmentioning

confidence: 99%

“…The association of FMF with other in ammatory diseases such as spondyloarthritis, immunoglobulin A vasculitis/Henoch-Schönlein purpura, polyarteritis nodosa, in ammatory bowel disease (IBD), chronic arthritis, and protracted febrile myalgia has been well de ned and it is suggested that FMF and associated in ammatory diseases should not be considered as the coexistence of different clinical entities [5][6][7][8]. Previous studies have reported uveitis, scleritis, episcleritis, optic neuritis, and frosted branch angiitis of retinal vein among ocular in ammatory manifestations of FMF [9][10][11][12][13][14]. Although FMF has been thought of as a predisposition to a variety of in ammatory processes, the possible association of FMF with ocular in ammatory diseases (OIDs) is not clear contrary to its known association with sacroiliitis, vasculitis, and IBD.…”

Section: Introductionmentioning

confidence: 99%

Ocular inflammatory diseases in children with familial Mediterranean fever: a true association or a coincidence?

Avar-Aydın

Aydın

Özçakar

et al. 2021

Preprint

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Purpose: To describe the characteristics of patients with familial Mediterranean fever (FMF) concurrent with ocular inflammatory disease (OID) and to criticize possible relations between them.Methods: Clinical data were extracted from electronic medical records. Additionally, the medical literature on OIDs in FMF was reviewed.Results: Among 512 pediatric patients with FMF, five patients were found to have OIDs: bilateral chronic uveitis, recurrent orbital myositis (ROM), recurrent optic neuritis, and acquired Brown’s syndrome. The first cases of ROM and acquired Brown’s syndrome in FMF have been introduced in the literature. All patients presented with early-onset typical FMF attacks carried at least one M694V mutation and experienced OID while on colchicine. Conclusion: Increased frequency of OIDs in FMF as per the pediatric population and relapsing and chronic course of OIDs occasionally with concurrent FMF attacks suggest that this inflammatory syndrome especially those carrying M694V mutations may be a predisposing factor for OIDs.

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“…The identification of underlying causes has important implications for therapy. Reported conditions that can present with secondary FBA include herpes simplex virus retinitis, varicella zoster virus retinitis, cytomegalovirus retinitis, toxoplasmosis, tuberculosis, familial Mediterranean fever, antiphospholipid antibody syndrome, systemic lupus erythematosus, syphilis, HIV infection, Behçet's disease, Hodgkin lymphoma (HL), and direct ocular invasion by malignant cells …”

Section: Introductionmentioning

confidence: 99%