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
Contents
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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