Definition
- Triad of rheumatoid arthritis, splenomegaly and neutropenia
Epidemiology
- Affects a small minority of RA patients, more often women
- Long standing, seropositive, nodular and erosive RA
- Strong association with HLA-DR4
Pathology
- Immune mediated neutrophil destruction with splenic sequestration
- Overlaps with large granular lymphocyte leukaemia
Clinical Features
- Recurrent bacterial infections, especially skin and respiratory
- Lymphadenopathy
- Skin pigmentation of the lower legs
- Chronic leg ulceration
- Hepatomegaly
- Weight loss and fever
- Joint disease may be inactive despite systemic features
Investigations
- Neutrophil count below 2 x 10^9 per litre
- Thrombocytopenia and haemolytic anaemia
- High titre rheumatoid factor and anti CCP antibodies
- Blood film and flow cytometry to exclude LGL leukaemia
Management
- Methotrexate as first line disease modifying therapy
- Rituximab for refractory disease
- G-CSF for severe neutropenia or infection
- Splenectomy for failed medical treatment, with recurrence of neutropenia possible
- Check neutrophil count before arthroplasty and discuss perioperative G-CSF with rheumatology
Complications
- Higher risk of surgical site and periprosthetic infection
- Poor wound healing in ulcerated or pigmented leg skin
Reviewed by Professor Phong Tran, Head of Orthopaedic Surgery, Western Health. Last updated 10 October 2026.