1/16. polyarteritis nodosa involving the hepatobiliary system in an eight-year-old girl with a previous diagnosis of familial mediterranean fever.polyarteritis nodosa (PAN) is a vasculitis of small- and medium-sized muscular arteries with deposition of immune complex in the vessel wall. Although gastrointestinal involvement is common, the symptomatic involvement of the hepatobiliary system is rare. An eight-year old female patient with a previous diagnosis of familial mediterranean fever (FMF) was hospitalized for right upper quadrant pain and fever. The thickened gall bladder wall by ultrasonography, called for exploration. Histopathological evaluations of the liver biopsy and gall bladder revealed PAN. Corticosteroid therapy was initiated and the patient recovered fully. This case represents one of the rarest forms of PAN in childhood.- - - - - - - - - - ranking = 1keywords = nodosa (Clic here for more details about this article) |
2/16. familial mediterranean fever and glomerulonephritis and review of the literature.familial mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent and self-limited attacks of fever usually accompanied by polyserositis. amyloidosis is its most common renal complication. A number of reports have shown vasculitic diseases such as polyarteritis nodosa and Henoch-Schonlein purpura affecting the kidney in FMF. Here we present a patient with FMF and membranoproliferative glomerulonephritis and analyze the data published on these two entities.- - - - - - - - - - ranking = 1.9292937518132keywords = polyarteritis, polyarteritis nodosa, nodosa (Clic here for more details about this article) |
3/16. familial mediterranean fever, inflammation and nephrotic syndrome: fibrillary glomerulopathy and the M680I missense mutation.BACKGROUND: familial mediterranean fever (FMF) is an autosomal recessive disease characterized by inflammatory serositis (fever, peritonitis, synovitis and pleuritis). The gene locus responsible for FMF was identified in 1992 and localized to the short arm of chromosome 16. In 1997, a specific FMF gene locus, MEFV, was discovered to encode for a protein, pyrin that mediates inflammation. To date, more than forty missense mutations are known to exist. The diversity of mutations identified has provided insight into the variability of clinical presentation and disease progression. CASE REPORT: We report an individual heterozygous for the M680I gene mutation with a clinical diagnosis of FMF using the Tel-Hashomer criteria. Subsequently, the patient developed nephrotic syndrome with biopsy-confirmed fibrillary glomerulonephritis (FGN). Further diagnostic studies were unremarkable with clinical workup negative for amyloidosis or other secondary causes of nephrotic syndrome. DISCUSSION: Individuals with FMF are at greater risk for developing nephrotic syndrome. The most serious etiology is amyloidosis (AA variant) with renal involvement, ultimately progressing to end-stage renal disease. Other known renal diseases in the FMF population include IgA nephropathy, IgM nephropathy, Henoch-Schonlein purpura as well as polyarteritis nodosa. CONCLUSION: To our knowledge, this is the first association between FMF and the M680I mutation later complicated by nephrotic syndrome and fibrillary glomerulonephritis.- - - - - - - - - - ranking = 1.9292937518132keywords = polyarteritis, polyarteritis nodosa, nodosa (Clic here for more details about this article) |
4/16. A case of familial mediterranean fever and polyarteritis nodosa complicated by spontaneous perirenal and subcapsular hepatic hemorrhage requiring multiple arterial embolizations.The association of familial mediterranean fever (FMF) and polyarteritis nodosa (PAN) has been well established. These patients have been reported to have an overall better prognosis than other PAN patients. Herein we report a patient with FMF and PAN who died of sepsis following a severe course of recurrent bleeding episodes which required multiple embolization attempts. The 39-year-old Turkish male presented with abdominal pain of 1-month duration. He had been diagnosed with FMF at the age of 24. On admission, he had pallor with general ill appearance. Rebound tenderness was obtained in the right upper abdominal quadrant. He had mild anemia, leukocytosis, thrombocytosis, and hypoalbuminemia. On the 2nd day of his admission, he developed hypotension with a rapid decline in hemoglobin level. Abdominal angiography showed multiple aneurysms in the branches of renal arteries, superior mesenteric artery, and hepatic arterial system including left renal infarct, suggesting PAN. He was put on high-dose steroids and oral cyclophosphamide. Despite medical treatment, he developed intense abdominal pain, hypotension, tachycardia, and a rapid fall in hemoglobin on four occasions. Active bleeding sites were embolized in two different angiography sessions. Although the patient experienced no more recurrent bleeding, he died of multiorgan dysfunction syndrome resulting from sepsis 6 weeks after admission. polyarteritis nodosa associated with FMF may follow a grave course despite immunosuppressive therapy. Arterial embolization should be considered in the presence of bleeding aneurysms in addition to immunosuppressive therapy.- - - - - - - - - - ranking = 9.846468759066keywords = polyarteritis, polyarteritis nodosa, nodosa (Clic here for more details about this article) |
5/16. polyarteritis nodosa in a case of familial mediterranean fever.We describe a 7-year-old boy with familial mediterranean fever (FMF) complicated by polyarteritis nodosa (PAN) with distinct angiographic findings. On admission, he had abdominal pain, arthralgia, and severe fibromyalgia. During hospitalization, he displayed maculopapular eruptions, high blood pressure, gastrointestinal bleeding, and persistent constitutional symptoms mimicking a vasculitic process, most probably PAN. Renal angiography showed a perfusion defect compatible with a renal infarction secondary to a vasculitic process. He responded well to pulse methylprednisolone therapy with colchicine. We emphasize the rare association of FMF and PAN and the non-aneurysmal angiographic signs of PAN.- - - - - - - - - - ranking = 2.7292937518132keywords = polyarteritis, polyarteritis nodosa, nodosa (Clic here for more details about this article) |
6/16. Variable expression of vasculitis in siblings with familial mediterranean fever.familial mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent and self-limited attacks of serosal inflammation with abdominal pain, chest pain, and arthritis usually accompanied by fever. Different vasculitides such as polyarteritis nodosa (PAN) and Henoch-Schonlein syndrome (HSS) may be associated with FMF. We report two sisters of a Turkish family with FMF who developed distinct vasculitides. The younger sister developed severe PAN with perirenal hematoma at the age of 13 years, the older sister presented with severe HSS and acute renal failure at the age of 19 years. Neither sister developed amyloidosis until the age of 30 years. This observation suggests that early events in the pathogenesis of PAN and HSS are generally quite similar.- - - - - - - - - - ranking = 1.9292937518132keywords = polyarteritis, polyarteritis nodosa, nodosa (Clic here for more details about this article) |
7/16. The efficacy of interferon-alpha in a patient with resistant familial mediterranean fever complicated by polyarteritis nodosa.familial mediterranean fever (FMF) is a recurrent self-limiting polyserositis. polyarteritis nodosa (PAN) complicating FMF is very rare. Here, we present a 17-year-old male patient with FMF who subsequently developed PAN 2 weeks after hepatitis A infection. This case was also complicated with perirenal haematoma, and right nephrectomy was performed. The clinical condition of the patient was improved after therapy with intravenous and oral corticosteroid and intravenous cyclophosphamide. However, the FMF attacks and vasculitic skin lesions again occurred while he was using colchicine plus immunosuppressive agents a few months later. interferon-alpha therapy was administered and the attacks were resolved within 3 months. He has not experienced any other symptom during the follow-up period of 28 months.- - - - - - - - - - ranking = 7.9171750072528keywords = polyarteritis, polyarteritis nodosa, nodosa (Clic here for more details about this article) |
8/16. Spontaneous renal laceration as the presenting feature of polyarteritis nodosa in a patient with familial mediterranean fever after hepatitis A infection.We report a life-threatening spontaneous renal laceration with no history of bleeding diathesis or any trauma in a patient with FMF after acute hepatitis a virus (HAV) infection. Right nephrectomy was inevitable and histological investigation of the removed right kidney revealed a polyarterits nodosa (PAN). This case underlines the possibility that simultaneous PAN and immunsupressive treatment besides colchicine should be considered for patients with FMF. Also, patients with FMF who are not immune may be vaccinated for HAV which could be a predisposing mechanism for vasculitic hemorrhage.- - - - - - - - - - ranking = 7.9171750072528keywords = polyarteritis, polyarteritis nodosa, nodosa (Clic here for more details about this article) |
9/16. polyarteritis nodosa and familial mediterranean fever: a report of 2 cases and review of the literature.Two cases of polyarteritis nodosa (PAN) in patients with familial mediterranean fever (FMF) are reported. These and another 11 cases found in the literature suggest that PAN occurs more commonly in patients with FMF than would be expected in the general population. Perirenal hematoma, which is surprisingly high in patients with FMF, is a life threatening complication of PAN. The diagnosis of PAN in patients with FMF may be delayed due to the similarity of the clinical manifestations of both diseases.- - - - - - - - - - ranking = 2.7292937518132keywords = polyarteritis, polyarteritis nodosa, nodosa (Clic here for more details about this article) |
10/16. Perirenal and renal subcapsular haematoma as presenting symptoms of polyarteritis nodosa.Two young men, were hospitalized due to acute massive blood loss with left abdominal flank pain. In both cases renal angiography showed signs of a haemorrhagic event in the left kidney, perirenal in one and subcapsular in the other. Microaneurysms indicated a diagnosis of polyarteritis nodosa, supported by renal biopsy in one case. Renal haemorrhage is an infrequent presentation of polyarteritis nodosa. Furthermore, one patient suffered also from familial mediterranean fever, and is the fifth reported case with this combination of diseases.- - - - - - - - - - ranking = 11.575762510879keywords = polyarteritis, polyarteritis nodosa, nodosa (Clic here for more details about this article) |
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