A Case of Familial Mediterranean Fever with Cutaneous Vasculitis and Immune Complex Nephritis: Light, Electron, and Immunofluorescent Study of Renal Biopsy

Schlesinger, M; Kopolovic, Juri; Viskoper, Reuven J.; Ron, Noemi

doi:10.1093/ajcp/80.4.511

Cited by 29 publications

(21 citation statements)

References 3 publications

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“…Given that, most of the FMF-associated vasculitis cases had MEFV gene mutations, contributing role of these mutations for vasculitis development may be considered in the pathogenesis. In line with this view, our literature search showed that most of the FMF patients reported in literature with coexistent HSV or PAN had [7,9,10,13,14,65,66] Medium vessel vasculitic diseases Polyarteritis nodosa [7, 8, 11-13, 15, 70, 71] Large vessel vasculitic diseases Unreported Unclassified vasculitic conditions Protracted febrile myalgia [16,60,[72][73][74] Behçet's disease [17,18,79] Cutaneous vasculitis [87,88] either homozygous or compound heterozygous M694V mutations. However, since many FMF patients carrying these mutations do not have associated vasculitis, we admit that this coexistence cannot solely be explained by the presence of MEFV gene mutations.…”

Section: Pioneering Reports About Fmf-vasculitis Associationmentioning

confidence: 66%

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Coexistence of vasculitides with Familial Mediterranean Fever

Aksu

Keser

2011

Rheumatol Int

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Familial Mediterranean fever (FMF) is the most common autoinflammatory disease characterized by recurrent self-limited attacks of fever accompanied with peritonitis, pleuritis, or arthritis. FMF may coexist with various systemic inflammatory diseases including vasculitides, spondyloarthritis, multiple sclerosis, and inflammatory bowel disease. Among these coexistences, this review concentrates on vasculitic disorders, with the aim of increasing the awareness of FMF-vasculitis association. This association does not merely show a coincidentally increased frequency of vasculitic disorders in FMF; rather, it seems that FMF patients might be at increased risk of developing vasculitis. Indeed, as also suggested by some authors, vasculitis might be an essential feature of FMF. Among the vasculitic disorders reported to be associated with FMF, Henoch-Schönlein purpura, and classical polyarteritis nodosa come the first, possibly followed up by protracted febrile myalgia. There is also an ongoing debate whether Behçet's disease (BD) more frequently seen in FMF than expected by chance alone. In this review, the associations of various vasculitic disorders with FMF and the possible pathogenic mechanisms underlying these associations, as well as the frequencies and clinical significances of FMF-related MEFV mutations in various vasculitides including BD, are discussed in the context of the available data.

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Section: Pioneering Reports About Fmf-vasculitis Associationmentioning

confidence: 66%

“…Various types of skin rashes including palpable purpura have also been reported in patients with FMF [87]. Among these skin rashes, only erysipelas-like erythema is pathognomonic for FMF.…”

Section: Cutaneous Vasculitismentioning

confidence: 99%

Coexistence of vasculitides with Familial Mediterranean Fever

Aksu

Keser

2011

Rheumatol Int

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“…The occurrence of circulating immune complexes in 50% of FMF patients, complement consumption, defective inhibition of complement activation (C5a), and uncontrolled release of tumor necrosis factor (TNF) support the view that an immune-related mechanism plays a role [64,65,66]. The presence of immunoglobulins, C3, and fibrinogen in skin and kidney biopsies of some patients are interesting features.…”

Section: Fmf-associated Vasculitidesmentioning

confidence: 92%

Familial Mediterranean fever

Bakkaloğlu

2003

Pediatric Nephrology

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Familial Mediterranean fever (FMF) is the most frequent periodic syndrome characterized by recurrent attacks of polyserositis. Fever, abdominal pain, chest pain, and arthritis/arthralgia are the leading symptoms. It is an autosomal recessive disorder, which primarily affects Jewish, Armenian, Turkish, and Arab populations. The FMF gene ( MEFV) has recently been cloned to chromosome 16p, which encodes pyrin. Genotype-phenotype correlation is not well established. Amyloidosis is the most severe complication of FMF. The SAA1-alpha/alpha genotype was associated with an increased risk of amyloidosis. Colchicine treatment not only decreases the frequency and severity of attacks, but also prevents amyloidosis. Certain vasculitides, namely Henoch-Schonlein purpura and polyarteritis nodosa, are more frequent among FMF patients.

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“…In 10 to 70% of cases, dermatological abnormalities are present; these consist most commonly of erysipelas-like lesions [6,12,46,47] which involve the legs, ankles, and dorsum of the feet. Other manifestations are nodular rashes, Schonlein-Henoch purpura [5,46,47], urticaria [6,47], bullous lesions [48], and a vasculitis which is usually associated with an immune-complex nephropathy [49]. In addition, splenomegaly is present in about 30% of cases overall [11][12][13] and, like the arthropathy and dermatological lesions, is less frequent in Arab patients [18].…”

Section: Clinical Aspectsmentioning

confidence: 99%

Recurrent Hereditary Polyserositis or Familial Mediterranean Fever: An Overview

Cook

1991

Ann Saudi Med

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The etiology and pathogenesis of recurrent hereditary polyserositis (RHP) remain undetermined. There is good evidence for a genetic basis (transmission being by a recessive Mendelian mode of inheritance), but not all cases have a family history and an infective (or other environmental) component has not been excluded. The marked variation in presentation and complications (in particular the prevalence of amyloid deposition) in different series suggests that this might not be a homogeneous entity. Vascular involvement is a basic prerequisite for the periodic attacks, and evidence for transient immunological abnormalities is incontrovertible. There are no reliable diagnostic techniques. Colchicine is of value in treatment, but its mode of action is unclear. More macro-and microepidemiological studies are required and will be of paramount importance. Clinical observations and molecular genetic studies should address the possibility that two or more immunological or metabolic defects might be interwoven; why for example do only some affected groups develop AA amyloidosis? Simple, non-invasive diagnostic tests are required for the uncomplicated disease and also for the presence of amyloid deposition. Preventive strategies might eventually involve genetic engineering techniques, but in the immediate future, education of doctors and other health care workers-to raise the "index of awareness" of RHP, in order that colchicine treatment can be commenced early in the disease-forms an important strategy. Colchicine therapy is not without complications and an alternative chemotherapeutic agent should be sought. GC Cook, Recurrent Hereditary Polyserositis or Familial Mediterranean Fever: An Overview. 1991; 11(5): 576-584 Many aspects of recurrent hereditary polyserositis (RHP) have recently been reviewed [1][2][3][4]. The first description of a familial condition comprising "recurring attacks of a peculiar nature" was made by Janeway and Mosenthal in 1908. In that report, a 16-year-old Jewish schoolgirl "without special neurotic inheritance" had suffered febrile bouts associated with abdominal pain, which consistedof prodromal, crescendo, and recovery phases, since the age of 2 weeks [5]. In view of the dramatic nature of many attacks, it is perhaps surprising that no records of the disease have been discovered in early medical (and also lay) literature from the Mediterranean and Middle East, including the work of Hippocrates, the Bible, and the Quran. Subsequent accounts of RHP followed

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A Case of Familial Mediterranean Fever with Cutaneous Vasculitis and Immune Complex Nephritis: Light, Electron, and Immunofluorescent Study of Renal Biopsy

Cited by 29 publications

References 3 publications

Coexistence of vasculitides with Familial Mediterranean Fever

Coexistence of vasculitides with Familial Mediterranean Fever

Familial Mediterranean fever

Recurrent Hereditary Polyserositis or Familial Mediterranean Fever: An Overview

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