Felty syndrome
| Classification according to ICD-10 | |
|---|---|
| M05.0- | Felty syndrome |
| ICD-10 online (WHO version 2019) | |
The Felty's syndrome is a complication of long-standing, mostly severe rheumatoid arthritis . It is rare and only occurs in about 1% of people with rheumatoid arthritis .
When defining Felty's syndrome, the following symptoms are required:
- Rheumatoid arthritis
- Splenomegaly and lymph node swelling
- Leukopenia (especially granulocytopenia)
In addition, the rheumatoid factor is highly positive.
clinic
In addition to the arthralgia, some of the patients with Felty syndrome suffer from increased infections. This is attributed to the lack of immune cells ( leukocytes ). In addition to pneumonia, skin infections of the lower legs also occur. In addition to the lack of immune cells, inflammation of small vessels ( vasculitis ) seems to be responsible here as part of the underlying rheumatic disease.
root cause
The cause of this disease is not yet fully understood. It is also unclear why some of the rheumatoid arthritis patients develop Felty's syndrome and others do not. There seems to be a malfunction in the area of blood cell formation, so that the - possibly even increased - formed white blood cells are retained in the spleen and are absent from the peripheral blood . Patients with this constellation have antibodies against components of white blood cells.
therapy
First, the underlying rheumatoid arthritis is treated (see there). Patients with severe infections are also treated locally and systemically for them (e.g. antibiotics ). In individual cases, cell growth factors (e.g. G-CSF = granulocyte colony stimulating factor) are also used. To prevent excessive white blood cells from being trapped in the spleen, the spleen is removed in severe cases ( splenectomy ). However, all of these measures are still at the research stage and the long-term results are still unknown.
swell
- H. Nagasawa, T. Takeuchi: Felty Syndrome In: Nippon Rinsho / Japanese Journal of Clinical Medicine. May 2005; 63 Suppl 5. pp. 281-284.
- GP Balint, PV Balint: Felty's syndrome. In: Best Practice & Research Clinical Rheumatology . October 2004; 18 (5). Pages 631-645.
- LC Hofbauer, J. Diebold and AE Heufelder: Rheumatoid arthritis, neutropenia and splenomegaly: The Felty Syndrome. In: German Medical Weekly December 8, 1995; 120 (49). Pages 1689-1694.
