Felty syndrome (FS), also known as ”super rheumatoid” disease, is a severe form of rheumatoid arthritis (RA), characterized by a triad of RA, splenomegaly and. Best Pract Res Clin Rheumatol. Oct;18(5) Felty’s syndrome. Balint GP(1), Balint PV. Author information: (1)Fourth General Rheumatology. 3 Sep Felty’s Syndrome. Aka: Felty’s Syndrome, Felty Syndrome Syndrome de Felty. Italian, Sindrome di Felty. Portuguese, Síndrome de Felty.
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Management and treatment FS is difficult to treat and the standard fe care is directed against underlying RA with an additional goal of treating neutropenia and recurrent infections.
Specialised Social Services Eurordis directory. Splenomegaly-neutropenia-rheumatoid arthritis syndrome Prevalence: Search other sites for ‘Felty’s Syndrome’. Recombinant granulopoietic growth factors G-CSF have also been used to improve neutrophil counts. Early presentation is commonly seen in the joints of hands and of the feet. Immunosuppressive therapy for RA often improves granulocytopenia and splenomegaly; this finding reflects the fact that Felty’s syndrome is an immune-mediated disease.
This increase sinddrome defense activities ultimately causes hypertrophy of the spleen, leading to splenomegaly.
Recurrent bacterial infections are mostly due to the severe, otherwise unexplained neutropenia.
When the spleen becomes enlarged, it is a strong sign of infection somewhere in the body, and can be caused by inflammatory conditions such as rheumatoid arthritis. Autosomal dominant disorders Syndromes affecting blood Inflammatory polyarthropathies Rare syndromes.
Neutrophils are the most abundant cells among white blood cells and play an important role in the eindrome system by destroying bacteria via phagocytosis. The spleen is a lymphatic organ, which means it is largely involved in the immune system and immune responses. Diagnosis can be confirmed by use of X-rays or synovial fluid analysis. A syndrome characterized sindorme the presence of rheumatoid arthritis, splenomegaly, and granulocytopenia.
Started inthis collection now contains interlinked topic pages divided into a tree of 31 specialty books and chapters. LGL expansion associated with uncomplicated RA is immunogenetically and phenotypically very similar to but clinically different from FS. Didn’t get the message? FS differs from RA by more severe arthritis and extra-articular manifestations. This page was last edited on 2 Novemberat Prognosis FS has a poor prognosis and recurrent infections may lead to increased mortality.
Patients should address specific medical concerns with their physicians. As a result of neutropenia, affected individuals are increasingly susceptible to certain infections. An acronym can be used to make recognizing this disease somewhat easier:. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. Heberden’s node Bouchard’s nodes. Add to My Eflty. It is commonly overlooked or misdiagnosed as other conditions e.
Disease or Syndrome T Inflammation Infectious Septic arthritis Tuberculosis arthritis Reactive arthritis indirectly. Clinical description FS is about 3 times more common in females during the third through fifth decades of life earlier in men. These autoantibodies or their immune complexes may sindrom stimulate neutrophils, thus completing a self-sustaining cycle that drives the depletion of mature neutrophils. Definition NCI A syndrome characterized by the presence of rheumatoid arthritis, splenomegaly, and granulocytopenia.
Generate a file for use with external citation management software. Back Links pages that link to this page. A decrease in the sinddome of neutrophils stimulates an autoimmune response which leads to arthritis.
Therefore, in order to decide upon and begin treatment, the cause and relationship of neutropenia with the overall condition must be well understood. National Center for Biotechnology InformationU.
FS is uncommon in the African American population. This condition can affect and damage several other body systems such as the eyes, heart, lungs, and blood vessels. Clinically it is characterized by severe joint destruction contrasting with moderate or absent joint inflammation and severe extra-articular disease, including a high frequency of rheumatoid nodules, lymphadenopathy, hepatopathy, vasculitis, leg ulcers, skin pigmentation etc.
FS is about 3 times more common in females during the third through fifth decades of life earlier in men. If this condition is not treated, the joint space will completely narrow, causing ankylosis.
Etiology The pathophysiology of FS is still not well understood butit has been proposed that an autoimmune response against neutrophil antigens might be the underlying mechanism.
A major challenge in treating FS is recurring infection caused by felth.
Felty’s syndrome – Wikipedia
Supplemental Content Full text links. The loss and destruction of neutrophils leading to neutropenia is therefore, inflammation-driven due to the body’s need for the immune response.
Microscopic analysis of spleen biopsies reveals enlargement of the germinal center in follicles, and infiltration of neutrophils and macrophages. There is no real treatment for Felty’s syndrome, rather the best method in management of the disease is to control the underlying rheumatoid arthritis.
As the disease progresses it can be seen in the knees, wrists, hips, and shoulders. Recurrent infections may lead to increased mortality.