IgA nephropathy in patients with familial Mediterranean fever

Two patients with a long-standing history of familial Mediterranean fever were found to have both microscopic hematuria and proteinuria during the acute attacks. Kidney biopsies from both patients revealed diffuse mesangial proliferative glomerulonephritis with intense mesangial IgA and C3 deposits and no evidence of amyloidosis. To our knowledge these are the first 2 cases documenting the presence of mesangial IgA nephropathy in patients with familial Mediterranean fever.     

Polyarteritis nodosa type vasculitis in a patient with familial Mediterranean fever treated with cyclosporin A

Patients with amyloidosis secondary to familial Mediterranean fever (FMF) are known to tolerate cyclosporin A poorly. We report a case of severe cyclosporin toxicity in a patient with FMF amyloidosis who underwent kidney transplantation. The clinical syndrome consisted of severe gastrointestinal, neuromuscular, and psychiatric disturbances. Histological examination of the transplanted kidney revealed vasculitis of the polyarteritis nodosa type. We hypothesize that FMF patients are more vulnerable to the acute vascular toxicity of cyclosporin due to defective inhibition of complement activation, leading to a widespread vasculitis of the polyarteritis nodosa type.     

Massive renal and adrenal calcifications in a young dialysis patient with familial Mediterranean fever

A 34-year-old patient was hospitalized for progressive developmentof asthenia, muscle weakness and weight loss. This Greek manhad familial Mediterranean     

Mesangial proliferative glomerulonephritis with Legionnaires' disease. A case report

Although the clinical and biochemical features of renal involvement in Legionnaires' disease have been well described, only a few case reports detailed the histological changes. A patient with Legionnaires' disease who developed acute renal failure is described; a biopsy specimen revealed mesangial proliferative glomerulonephritis. The renal morphological changes in Legionnaires' disease are reviewed.     

Familial Mediterranean fever and mesangial proliferative glomerulonephritis: report of a case and review of the literature

In familial Mediterranean fever (FMF), a genetically inherited disease characterized by fever and serositis, renal involvement is mainly AA amyloidosis. We report a patient with FMF who developed mesangial proliferative glomerulonephritis; presumably in response to colchicine treatment, the activity of the disease decreased and renal function tests and urinary findings normalized. This report emphasizes the concurrent existence of mesangial proliferative glomerulonephritis with FMF in the absence of renal amyloidosis. Due to increased inflammatory response observed in FMF, immunologic glomerular injury, a common cause of glomerulonephritis, may occur more frequently in patients with FMF.     

Rapid progressive glomerulonephritis in patients with familial Mediterranean fever

Two patients with a long-standing history of familial Mediterranean fever (FMF) presented with gross hematuria, oliguria, and acute renal failure; both required dialysis support. Kidney biopsies from both patients revealed crescentic rapid progressive glomerulonephritis (RPGN) without amyloidosis. One patient recovered renal function with methylprednisolone pulse therapy and cyclophosamide. The second patient did not improve and required regular hemodialysis. He is asymptomatic on colchicine therapy. To our knowledge, these are the first cases documenting the presence of RPGN in patients with FMF.     

A case of familial Mediterranean fever with amyloidosis as the first manifestation

We describe a 22-year-old Turkish woman with nephrotic syndrome who had a history of acute myelocytic leukemia. After careful clinical evaluation, the patient underwent a renal biopsy. Light microscopic examination showed deposition of Congo-positive material both in the mesangium and around the small vessels. By histochemical analyses, the deposited material was proved to be amyloid A (AA). Because the patient's history did not reveal any event that might explain the development of secondary amyloidosis, she was screened for mutations causing familial Mediterranean fever (FMF) and was found to be homozygous for the M694V mutation by denaturing gradient gel electrophoresis. We recommend that FMF-Phenotype II and the development of amyloid nephropathy, before or without other symptoms of FMF, should be kept in mind in the face of unexplained proteinuria/amyloidosis, especially in high-risk ethnic groups. © 2001 by the National Kidney Foundation, Inc.     

Familial Mediterranean fever and membranous glomerulonephritis: report of a case

Familial Mediterranean fever (FMF) is an autosomal recessive genetic disease characterized by recurrent attacks of fever and painful episodes of sterile polyserositis. Kidney involvement may occur as a result of secondary amyloidosis during the course of FMF. Previously, different types of glomerulopathies such as IgM and IgA nephropathy, crescentic glomerulonephritis, diffuse proliferative glomerulonephritis, minimal change disease, and membranoproliferative glomerulonephritis were rarely reported. We herein represent a first case of membranous glomerulonephritis who had complete remission with colchicine treatment in the course of familial Mediterranean fever.     

Pseudo-acute abdomen in familial Mediterranean fever. A case report]

The Authors report a case of familial mediterranean fever with pseudo-acute abdomen recently observed and emphasize how a careful anamnesis can avoid unnecessary surgical intervention.     

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.     

黄芪多糖治疗大鼠系膜细胞增生性肾炎的实验研究

目的:探讨黄芪多糖(APS)对大鼠系膜增生性肾小球肾炎(MsPGN)模型病理改变及尿和血中白介素6(IL-6)的影响,寻找黄芪多糖治疗MsPGN的机理。方法:采用抗Thy-1抗体建立SD大鼠的MsPGN模型,再通过灌服黄芪多糖,观察大鼠MsPGN模型病理改变、尿和血中的IL-6的变化。结果:大鼠MsPGN模型尿和血中IL-6的含量较正常大鼠明显增高。黄芪多糖能明显改善大鼠MsPGN的蛋白尿症状,能明显降低大鼠MsPGN尿和血中IL-6的含量,能明显抑制系膜细胞(MC)的增生和基质的增多,并能减少免疫复合物的沉积,与雷公藤多甙比较无显著差异(P>0.05)。结论:(1)黄芪多糖能明显改善MsPGN大鼠蛋白尿;(2)黄芪多糖能降低尿和血中IL-6的含量;(3)黄芪多糖能抑制系膜细胞的增生和基质的增多;(4)黄芪多糖可能通过抑制IL-6的分泌而抑制系膜增生。     

The coexistence of familial Mediterranean fever and polyarteritis nodosa; report of a case

We describe a 14-year-old boy with a 5-year history of familial Mediterranean fever (FMF), treated with colchicine, who developed polyarteritis nodosa (PAN). He was admitted to our hospital with fever, general weakness, arthritis, and purpura. Five weeks after admission, hypertension was noted. Skin biopsy showed perivascular leukocyte infiltration in the epidermis. An aortography revealed multiple aneurysms of the renal, common hepatic, and intercostal arteries. He was treated with intravenous methylprednisolone, oral cyclophosphamide, and azathioprine. The known rare association of FMF and PAN is discussed. Received June 14, 1995; received in revised form January 26, 1996; accepted February 9, 1996     

Polyglandular endocrine failure in a patient with amyloidosis secondary to familial Mediterranean fever

Familial Mediterranean fever (FMF) is 1 of the major causes of secondary amyloidosis. Renal involvement is the main clinical complication and it mostly presents with nephrotic syndrome and chronic renal failure. Although deposition of amyloid has been reported in several endocrine glands such as the adrenal, thyroid, and testes, clinically significant functional impairment is uncommon. Herein, we describe a patient in whom the diagnosis of FMF was based on molecular screening and who presented with recurrent hypoglycemic attacks and extensive amyloid deposition affecting various organ function including adrenal, thyroid, parathyroid, testes, intestinal system, and the heart. © 2001 by the National Kidney Foundation, Inc.     

Myositis in a patient with familial Mediterranean fever and spondyloarthritis successfully treated with anakinra

Familial Mediterranean fever is an autosomal-recessive autoinflammatory disorder more commonly observed in Mediterranean populations and characterized by recurrent episodes of fever, serositis, myalgia and arthritis. There is rarely any association with spondyloarthritis. The most important long-term complication is progressive systemic type AA amyloidosis. Treatment with colchicine is effective in reducing the frequency of attacks and prevents the development of amyloidosis. However, 5% of cases are considered resistant to colchicine. We here describe the case of a 39-year-old man, with a history of arthritis, arthralgias, and sacroiliitis in the course of a familial Mediterranean fever. He is homozygous for the M694I mutation in the MEFV gene. He subsequently developed myositis of the right quadriceps muscle confirmed by magnetic resonance imaging, electromyography and histology. He had frequent and severe arthralgias, despite colchicine, then etanercept and adalimumab, impairing his quality of life. The patient was successfully treated with the IL-1 receptor antagonist anakinra with a dramatic improvement of muscular and articular symptoms. To our knowledge, our patient is the first patient with coexisting FMF, spondyloarthritis and myositis responding to anakinra treatment. Moreover this is the second case in the literature of myositis associated with familial Mediterranean fever.