Felty’s syndrome

Felty s syndrome An extra-articular manifestation of rheumatoid arthritis associated with splenomegaly and neutropenia. 

The autoimmune rheumatic diseases consists of Rheumatoid Arthritis (RA), Spondylarthritis (SpA), Systemic Lupus Erythematosus (SLE), Polymyositis, Dermatomyositis, Polymyalgia Rheumatica, Acute Temporal Arteritis, Giant Cell Arteritis, Behcet s Disease, Sjorgren s Syndrome, Felty s Syndrome and Mixed Connective Tissue Disease (MCTD). Spondylarthritis (SpA) can be subdivided in Reactive Arthritis (ReA), Ankylosing Spondylitis (AS), Psoriatic Arthritis (PsA), Arthritis associated with the inflammatory bowel diseases are Crohn s disease and Ulcerative Colitis (IBD), Undifferentiated SpA (UspA) and Sacro-ilitis, Juvenile SpA and Acute Anterior Uveitis (AAU). 

A14. Andreiss, M., Hurd, E. R., Lospalluto, J., and Ziff, M., Comparison of the presence of immune complexes in Felty s syndrome and rheumatoid arthritis. Arthritis Rheum. 21, 310-315 (1978). 

Rheumatoid arthritis is a chronic, recurrent systemic inflammatory disorder that primarily affects the joints. The incidence is typically between 1% and 3% of the UK population with a female preponderance of 3 1. Age of onset is typically between the ages of 20 and 40 years. The small joints of the hands and feet are usually affected first and presentation is usually symmetrical. Deformities may arise and the condition may progress to larger joints. In the most severe of cases, extra-articular tissues may be affected, including the lungs, muscle tissues and blood vessels. Additional complications may include Sjdgren s syndrome and Felty s syndrome. 

Felty s syndrome, lupus erythematosus, Reiter s disease, Still s disease, Wegener s disease, histiocytosis, etc. 

Felty s syndrome (A.R. Felty, 1924) is a special form of seropositive polyarthritis. It leads to considerable changes in the articular structures. Characteristic findings include splenomegaly, leucopenia, lymphadenopathy and skin pigmentation. Men are mainly affected. The liver is involved in about 70% of patients. Transaminases and y-GT values are moderately elevated, and there is an occasional rise in alkaline phosphatase. Lymphocytic infiltrates of the sinusoids, proliferation of Kupffer cells and periportal fibrosis are detectable histologically. Nodular regenerative hyperplasia can develop, which may subsequently cause portal hypertension and macronodular cirrhosis. (71, 95, 104)... 

Blendis, LJM., Lovell, D., Barnes, C.G., Ritland, S., Cattan, D. Oesophageal variceal bleeding in Felty s syndrome associated with nodular regenerative hyperplasia. Ann. Rheum. Dis. 1978 37 183—186... 

Eosinophilia can occur in patients receiving long-term G-CSF for congenital neutropenia (6). Patients with congenital neutropenia or Felty s syndrome may also be susceptible to anemia and/or thrombocytopenia (6,40). 

Transient bone pain is common with GM-CSF. Prompt reactivation or worsening of rheumatoid symptoms has been observed in several patients with Felty s syndrome and in one seropositive rheumatoid arthritic patient (SED-13, 1113) (46,47). GM-CSF-induced acute IL-6 release and an increase in acute phase proteins were thought to be involved. 

Hazenberg BP, Van Leeuwen MA, Van Rijswijk MH, Stern AC, Vellenga E. Correction of granulocytopenia in Felty s syndrome by granulocyte-macrophage colony-stimulating factor. Simultaneous induction of interleukin-6 release and flare-up of the arthritis. Blood 1989 74(8) 2769-70. 

Rheumatoid arthritis in association with splenomegaly and neutropenia is known as Felty s syndrome. Thrombocytopenia also may be a manifestation of the syndrome. Patients with Felty s syndrome and severe leukopenia are more susceptible to infection. The decrease in granulocytes appears to be mediated by the immune system because splenectomy does not result in improvement of the patient. ... 

Thrombocytosis is another common hematologic finding with active rheumatoid arthritis. Platelet counts rise and fall in direct correlation with disease activity in many patients. Thrombocytopenia may result from toxicity of gold salts, penicillamine, or immunosuppressive therapy. Thrombocytopenia also may be observed in Felty s syndrome or vascuhtis. 

Although leukopenia is associated with Felty s syndrome, it also may result from toxicity of gold, sulfasalazine, penicillamine, immunosuppressive drugs, and biologic agents. Leukocytosis is seen commonly as a result of corticosteroid treatment. 

Birnie, GG, Eadie AS, Hosie CJ et al (1982) " in-labelled white blood cells in the diagnosis of Felty s syndrome. J Clin Pathol 35 74-76... 

Goetzl, E. J., 1976, Defective responsiveness to ascorbic acid of neutrophil random and chemotactic migration in Felty s syndrome and systemic lupus erythematosus, Ann. Rheum. Dis. 35 510. 

---