Felty's syndrome. MRI images show large spleen (splenomegaly). Radiol Res Pract. 2013; Palas J1, Matos AP1, Ramalho M1. Open

Feltys Syndrome ICD-10: M05.0

Definition

Felty's syndrome is a complication of arthritis (rheumatoid arthritis, RA) where extra-articular organs are attacked. The disease was first described by Felty in 1924 and has many common features LGL leukemia.

• The syndrome is characterized by low white blood cell count (leukopenia og neutropenia) and enlarged spleen (splenomegaly).

Occurrence

Felty's syndrome occurs in 1-2% of those with rheumatoid arthritis (RA).

Symptoms

Severe, “seropositive” RA

• Rheumatic nodules / nodules is common (89%)
• secondary Sjögren's syndrome detected at 70%
• Infections (related to low white blood cell count)

Medical investigations

• Spleen: Moderately enlarged (over 12 cm by ultrasound examination / CT / MR)
• Blood: Low white blood cell count (leukopenia og neutropenia)
  • neutropenia; Moderate (0,5-1,0) - severe (below 0,5)
• Splenomegaly enlarged spleen
• Almost everyone is RF and CCP (ACPA) positive in blood tests

Complications

Infections occur due to low numbers neutrophil white blood cells (neutropenia)

Treatment

Disease-reducing drugs for active rheumatoid arthritis (RA).

• Neupogen (filgrastim) is used to increase the number of neutrophils (reference: Lazaro E 2015)
• Splenectomy (surgical removal of the spleen) was previously standard treatment, but is rarely done now

Medical Prognosis / Life expectancy

Previously, the disease has had high mortality (25% over 5 years), but the prognosis has improved with newer treatment methods.

Incorrect diagnosis? (Similar diseases / differential diagnoses)

• Amyloidosis
• Chronic infection
• Cirrhosis of the liver
• LGL syndrome
• Malignancy (cancer)
• Medication-induced symptoms
• Sarcoidosis

Literature

• Lilleby V, 2000 (Norwegian Legeforeningen)
• Ovlia BM, 2014
• Balint GP 2004
• Grans Compendium in Rheumatology

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