1/41. reflex sympathetic dystrophy arising in a patient with familial mediterranean fever.A 14-year-old girl with familial mediterranean fever (FMF) had had acute attacks of fever, abdominal pain, and arthritis for 4 years. Her last arthritis attack was protracted, leading to reflex sympathetic dystrophy (RSD) in her right lower extremity. Physical therapy along with sympathetic ganglion block and corticosteroid therapy was used for the treatment. To our knowledge, this is the first reported case of RSD arising in a patient with FMF. Early recognition of RSD in FMF patients is important, and physical therapy should be applied along with medical treatment.- - - - - - - - - - ranking = 1keywords = arthritis (Clic here for more details about this article) |
2/41. Protracted familial mediterranean fever arthritis.familial mediterranean fever (FMF) is an inherited disorder characterized by recurrent attacks of fever and abdominal, chest, and articular pain. The articular attack of FMF is typically an acute, self-limited, large joint monoarthritis most often affecting the knee or hip. Rarely, a more protracted arthritis may occur. We describe two unusual cases of long-standing FMF arthritis with excellent response to synovectomy.- - - - - - - - - - ranking = 3.5keywords = arthritis (Clic here for more details about this article) |
3/41. sacroiliitis in familial mediterranean fever: an unusual presentation in childhood.familial mediterranean fever (FMF) is an autosomal recessively transmitted disease characterized by attacks of fever and serositis. The course of arthritis, which is a common manifestation of FMF, is generally benign. sacroiliitis due to FMF has been reported by several authors, but all the patients described so far had roentgenographic abnormalities, and most of them were adult cases. Here we report the youngest FMF patient with sacroiliitis without any abnormality on sacroiliac x-ray. She is also the first FMF patient in whom sacroiliac involvement was diagnosed by computed tomography (CT) in childhood. It is concluded that CT is a useful technique for the early diagnosis of destructive arthritis in FMF patients even in early childhood.- - - - - - - - - - ranking = 1keywords = arthritis (Clic here for more details about this article) |
4/41. Subtalar arthritis as a presenting symptom of familial mediterranean fever: case report and literature review.familial mediterranean fever (FMF) is an autosomal, recessive disease affecting mainly people of Mediterranean origin. The primary pattern of FMF is acute, self-resolving periodic attacks of high-grade fever, accompanied by either peritonitis, pleuritis, or arthritis and sometimes typical ankle rash that simulates erysipelas. Rare manifestations, such as pericarditis or massive knee effusion, have been reported in the literature as a presenting symptom of FMF. The final diagnosis has recently become more accurate by identification of the gene for FMF. We describe a unique presenting symptom of subtalar arthritis with no former personal or family history of FMF. A genetic evaluation revealed a 694/726 genetic variant that confirmed the diagnosis of FMF. Treatment with daily colchicine, 1 mg/day, resulted in complete resolution of all complaints.- - - - - - - - - - ranking = 3keywords = arthritis (Clic here for more details about this article) |
5/41. End-stage renal disease associated with familial mediterranean fever.A 39-year-old man had been suffering from periodic fever since childhood. He was started on hemodialysis due to secondary amyloidosis on December 2000. The patient was believed to have familial mediterranean fever (FMF) because of recurrent fever with peritonitis, arthritis and inflammatory changes and secondary amyloidosis in his kidneys, heart and colon. No other family member had recurrent fever. IL-6, TNF, and dopamine beta-hydroxylase were not increased in the febril phase. The patient was homozygous for the M6941 mutation. We report the first Japanese case of FMF associated with amyloidosis and confirmed by a gene mutation.- - - - - - - - - - ranking = 0.5keywords = arthritis (Clic here for more details about this article) |
6/41. Recurrent urticaria as a rare manifestation of familial mediterranean fever.familial mediterranean fever (FMF) is a genetic disorder characterized by acute episodes of fever with some combination of severe abdominal pain, pleurisy, arthritis, and skin rash. The case of a patient with recurrent urticaria referred for study of drug allergy is presented. After allergy had been ruled out, the urticaria was attributed to previously undiagnosed symptoms of an underlying systemic disease: FME. urticaria is the least frequent cutaneous manifestation of this disease, and genetic analysis was required to confirm the diagnosis.- - - - - - - - - - ranking = 0.5keywords = arthritis (Clic here for more details about this article) |
7/41. Allogeneic bone marrow transplantation: cure for familial mediterranean fever.We describe data on a 7-year-old girl with congenital dyserythropoietic anemia (CDA), who also had familial mediterranean fever (FMF). Repeated transfusions required since the age of 6 months to treat her CDA led to iron overload and a persistently high ferritin level. Her relapsing FMF made effective iron chelation therapy very difficult. Consequently, at the age of 4 years, she underwent allogeneic, sibling bone marrow transplantation (BMT). During conditioning for her BMT, symptoms of FMF, including splenomegaly, arthritis, and recurrent abdominal pain, began to resolve and she was gradually weaned off colchicine. Now, 2 years after the transplantation, she remains free from FMF symptomatology and is off all immunosuppressants. This case demonstrates that symptoms of FMF can be alleviated by the therapy used during allogeneic BMT. In this patient it is likely that the missing factor in FMF is now being provided by granulocytes derived from the stem cells within transplanted bone marrow.- - - - - - - - - - ranking = 0.5keywords = arthritis (Clic here for more details about this article) |
8/41. A new mutation causing autosomal dominant periodic fever syndrome in a Danish family.We describe four members in a family of 8 individuals over 3 generations with the autosomal dominant inherited periodic fever syndrome tumor necrosis factor receptor-associated periodic syndrome (TRAPS). The patients had recurrent episodes of fever, abdominal pain, arthritis, and rash. We examined the gene coding for the tumor necrosis factor receptor TNFRSF1A in all first-degree family members. In all 4 symptomatic members of the family, a hitherto undescribed mutation C98Y (380G-->A) in the TNFRSF1A gene was identified. In contrast, this mutation was not found in the 4 family members reported to be healthy nor in 50 normal control patients. The youngest member of the family, a 2-year-old boy, was treated successfully with etanercept.- - - - - - - - - - ranking = 0.5keywords = arthritis (Clic here for more details about this article) |
9/41. Update on treatment of Marshall's syndrome (PFAPA syndrome): report of five cases with review of the literature.Marshall's syndrome or PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is a recently described pediatric periodic disease characterized by recurrent febrile episodes associated with head and neck symptoms. The origin of this syndrome is unknown, and it can last for several years. During healthy periods, patients grow normally. The differential diagnosis includes other diseases characterized by periodic fevers, such as familial mediterranean fever, familial Hibernian fever, hyperglobulinemia D syndrome, Behcet's disease, cyclic neutropenia, juvenile rheumatoid arthritis, and several infectious diseases. Many treatments have been used, with various results, including antibiotics, nonsteroidal anti-inflammatory drugs, acetylsalicylic acid, colchicine, antiviral medicines, steroids, cimetidine, and tonsillectomy. We describe 5 new patients affected by PFAPA syndrome who were observed at the Department of Pediatric Otorhinolaryngology, Spedali Civili, Brescia, italy, from November 2000 to August 2001. All children underwent physical examination, bacterial, fungal, and viral cultures, chest radiography, and several laboratory studies. The patients were treated by successful tonsillectomy, and after a mean follow-up of 10 months, no recurrence was observed. An analysis of the literature is also presented with particular emphasis on the differential diagnosis of this rare illness and the results of the different therapeutic options.- - - - - - - - - - ranking = 0.5keywords = arthritis (Clic here for more details about this article) |
10/41. The arthritis of familial mediterranean fever.familial mediterranean fever (FMF) is a disease of unknown etiology and pathogenesis. In addition to fever, arthritis is among its most frequent manifestations. The arthritis of FMF is typically an acute, episodic, self-limited process with no sequelae. The radiographic features of FMF arthritis are usually limited to transient, often severe osteoporosis. synovial fluid analysis many mimic septic arthritis with very high white blood cell counts; cultures are uniformly negative. The course of FMF is almost always benign, with no residual articular incapacity. Some patients, limited to certain ethnic groups, develop renal amyloidosis. colchicine therapy modifies the natural history of the disease by decreasing the attack frequency and preventing amyloid deposition. At present, a lipocortin deficiency appears to be the likely candidate for a pathogenic mechanism. An unusual case with dramatic periarticular features (periostitis) and a protracted course with an excellent response to synovectomies is reported here. There is no explanation for the exuberant periarticular bone formation noted in this case, but a variety of recently discovered growth factors may be implicated.- - - - - - - - - - ranking = 4keywords = arthritis (Clic here for more details about this article) |
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