Felty’s syndrome

Felty’s syndrome

Felty’s syndrome also called Felty syndrome,  is a complication of long-standing and it is characterized by the combination of:

  • Rheumatoid arthritis,
  • Splenomegaly,
  • Neutropenia.

Signs of Felty’s syndrome:

  • Splenomegaly
  • Hepatomegaly
  • Lymphadenopathy
  • Rheumatoid Arthritis joint deformities ± active synovitis causing joint swelling and tenderness.
  • Anemia
  • Eye burning or discharge

 

Symptoms of Felty’s syndrome:Felty's syndrome

  • Fatigue
  • Anorexia
  • Weight loss
  • Recurrent infections due to neutropenia (lung and skin infections are most common).
  • Left upper quadrant pain (due to splenomegaly or splenic infarcts).
  • Symptoms related to Rheumatoid arthritis, including joint swelling, pain, stiffness and deformity.

Mnemonic:

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Felty's syndome mnemonic-medical institution

Epidemiology:

Felty’s syndrome affects approximately 1-3% of all patients diagnosed with RA, and RA occurs in about 1% of the general population. It appears to be rare in children and the African American population. The true prevalence of FS is difficult to ascertain because many affected patients are asymptomatic. Prevalence may be decreasing with the advent of more potent antirheumatic agents. Few data suggest that the international frequency of FS differs significantly from the US frequency.

FS is most common during the fifth through seventh decades of life and is usually associated with more than 10 years of preceding RA activity. Men are affected with FS earlier in the course of RA than women are. FS is about 3 times more common in females, though underreporting and asymptomatic cases hinder determination of the true sex ratio. FS is most common in whites and is uncommon in blacks. The HLA-DR4 genotype, a marker for more aggressive RA and more frequent extra-articular manifestations in whites, is strongly associated with FS.

Investigations and diagnosis of Felty’s syndrome:

– Blood tests:

  • Full blood count – for neutropenia ± anaemia of chronic disease
  • Autoantibodies: 95% of patients with Felty’s syndrome are positive for rheumatoid factorAnti-cyclic citrullinated peptide (anti-CCP) antibody  which has  high specificity for RA).
  • Inflammatory markers: ESR and C-reactive peptide.
  • Liver function tests – may be raised if there is liver involvement.

– Radiology:

  • Ultrasound or CT scan to evaluate splenomegaly.

– Bone marrow biopsy:

  • May be required to differentiate Felty’s syndrome from other diseases such as large granular lymphocyte syndrome or low-grade non-Hodgkin’s lymphoma.

 

Management of Felty’s syndrome:

  • Methotrexate is usually first choice
  • Hydroxychloroquine,
  • Ciclosporin,
  • Leflunomide ± methotrexate,
  • Gold,
  • Sulfasalazine,
  • Cyclophosphamide,
  • Rituximab ± methotrexate,
  • Splenectomy: Generally reserved for those not responding to medical treatment. Splenectomy improves neutropenia in most patients (80%).
  • Immunisation against influenza and pneumococcus

 

References:
http://www.patient.co.uk/doctor/Felty’s-Syndrome.htm
http://emedicine.medscape.com/article/329734-overview#a0156
http://www.nlm.nih.gov/medlineplus/ency/article/000445.htm
http://en.wikipedia.org/wiki/Felty’s_syndrome

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