Wednesday, June 1, 2011

Felty Syndrome

Felty syndrome is a potentially dangerous pathological condition associated with seropositive rheumatoid arthritis. It is characterized by the triad of rheumatoid arthritis, splenomegaly and granulocytopenia. Although many patients with Felty syndrome are asymptomatic, some develop severe infections secondary granulocytopenia.
Felty syndrome affects 1-3% of patients with rheumatoid arthritis and rheumatoid arthritis occurs in 1% of the population. True prevalence of the syndrome can not be determined because of asymptomatic nature. Felty syndrome is rare in children. Syndrome prevalence decreased by using potent antireumaticelor. Felty syndrome is unusually common in blacks and whites. Human leukocyte antigen DR4 marks aggressive rheumatoid arthritis and extra-articular manifestations common, associated with Felty syndrome.
Although many individuals with Felty syndrome are asymptomatic, others develop progressive and life threatening infections. Skin and lung infections are common. The level of disability in rheumatoid arthritis, immunosuppression caused by rheumatic with Felty syndrome affects mortality and morbidity of these patients.Felty syndrome is most common between the fifth and seventh decade of life. The condition is associated with a 10-year evolution of rheumatoid arthritis. Men are affected by the syndrome earlier than women.
The syndrome can be controlled Flety rheumatoid arthritis.Immunosuppressive therapy for this improves granulocytopenia and splenomegaly, reflecting the fact that Flety syndrome is immune mediated disease. Most traditional medicines are also useful in rheumatoid arthritis Flety syndrome.
Pathogenesis and causesAlthough the pathogenesis of Felty syndrome is not fully understood, points were demonstrated splenic sequestration and destruction of granulocytes secondary. Studies have shown low numbers of granulocytes in the splenic veins compared with the splenic artery. Immune complexes were linking granulocytes, decrease the growth factor granulocyte and numerous circulating autoantibodies against granulocytes. T lymphocyte leukemia shows great similarities with pathophysiological Felty syndrome.
Risk factors for development of Felty syndrome include:-Positivity for rheumatoid factor in high titersLong-duration disease-Aggressive and erosive synovitisPositivity for HLA-DR4 and DR4 homozigotismulExtra-articular manifestations of the disease.
Signs and symptoms associated with Felty syndromeFelty syndrome is most common between the fifth and seventh decade of life. The condition is associated with a 10-year evolution of rheumatoid arthritis. Men are affected by the syndrome earlier than women.The two conditions are separated by many years the onset.Occasionally, rheumatoid arthritis and Felty syndrome occur simultaneously. Extra-articular manifestations of rheumatoid arthritis: reuumatoizi nodules, pleuropericardita, vascultita, peripheral neuropathy, episclerita, Sjogren's syndrome, adenopathy, skin ulcers are more common in patients who develop Felty syndrome.Felty syndrome, accusing moderate symptoms of disease caused by inflammatory joint synovitis. Patient history shows a long period of active and aggressive articular disease.Flety syndrome, bacterial infections of the skin shows and respiratory tract. An aggressive immunosuppression directed at rheumatoid arthritis succeptibilitatea background contributes to the infection. Patients may experience pain in the left quadrant abdominal distension initiated or capsular infarctions spleen.The physical examination includes:-Splenomegaly, mild hepatomegaly, lymphadenopathyWeight-loss, rheumatoid nodules, Sjogren's syndromeTypical joint deformities, rheumatoid arthritis, synovitis-Vasculitis with ulcers of extremitiesBrown pigmentation, and palpable purpura of the extremities, myocardial periunghiale-Signs of systemic vasculitis: mononeurita multiplex extremity ischemia-Pleuritis, peripheral neuropathy, episclerita, portal hypertension.Evolution Felty syndrome:Granulocytopenia is defined as the absolute number of neutrophils in 2000/μL and an increased risk of infection as the number decreases. The incidence of infection increases significantly when polimorfonuclearele are under 1000/μL. Lymphoproliferative cancers are more prevalent in Felty's syndrome, especially non-Hodgkin's lymphomas.Although many individuals with Felty syndrome are asymptomatic, others develop progressive and life threatening infections. Skin and lung infections are common. The level of disability in rheumatoid arthritis, immunosuppression caused by rheumatic with Felty syndrome affects mortality and morbidity of these patients

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