Felty’s syndrome: diagnosis, treatment and lifestyle changes

Written by: Dr Gerald Coakley
Published: | Updated: 12/04/2023
Edited by: Robert Smith

Felty's syndrome is a rare disease that affects the body’s ability to fight infection. Whilst it is sometimes a complication of rheumatoid arthritis, the exact cause remains unknown.

 

worried woman with feltys syndrome

 

We spoke with leading rheumatologist, Dr Gerald Coakley, to find out how Felty’s syndrome is diagnosed as well as what treatment options and lifestyle changes can help to relieve symptoms.  

 

What is Felty's syndrome?

Felty’s syndrome is an uncommon complication of rheumatoid arthritis, first described by the American Physician A.R. Felty. The most important aspect of the condition is a low neutrophil count. Neutrophils are one of several types of white cell that circulate in the blood and are key to preventing and fighting bacterial infections. In 1924, Felty described five patients with rheumatoid arthritis, a low neutrophil count and an enlarged spleen (an organ in the left upper abdomen that is important in the healthy function of the immune system). It affects around 1% of people with rheumatoid arthritis.

 

What are the symptoms of Felty's syndrome?

The condition affects about twice as many females with rheumatoid arthritis as males, and usually develops 10 - 20 years after the onset of arthritis. So, most people who develop Felty’s syndrome are in their 50s or older. Other common features include rheumatoid nodules, weight loss, or Sjögren’s syndrome, giving rise to dry eyes and dry mouth. About one third of patients suffer from leg ulcers caused by skin vasculitis, or interstitial lung disease (fibrotic, scarring disease of the lung), singly or in combination.


How is Felty’s syndrome diagnosed?

Felty’s syndrome is first suspected when a patient with known rheumatoid arthritis develops a persistently low neutrophil count. This feature can be caused by many other conditions apart from Felty’s syndrome, including some of the therapies commonly used to treat rheumatoid arthritis (like Methotrexate, Leflunomide or Sulfasalazine), and several types of bone marrow disease.

 

The first step is usually to stop any medication that might be responsible for causing a low neutrophil count (a lot of treatments for rheumatoid arthritis can in fact cause a low count). If the count recovers, it usually means the medication was causing the problem and a switch to another treatment is a sensible step. If the count remains low, we usually arrange an abdominal ultrasound scan to look for an enlarged spleen, and refer to a haematologist (blood specialist) who will carry out a bone marrow biopsy. This gives us an accurate picture of the health of the bone marrow, and helps to exclude other serious causes of a low neutrophil count apart from Felty’s syndrome.

 

If it is shown that a drug is not responsible and the bone marrow shows no other cause of a low neutrophil count in a patient with proven rheumatoid arthritis, then that confirms the diagnosis of Felty’s syndrome. Most, but not all, patients will have an enlarged spleen.


How is Felty’s syndrome treated?

Felty’s syndrome has always been quite a rare disease, and it has become rarer still as our treatment of rheumatoid arthritis has become more effective in the last 20 years. That’s a great sign of progress, but it does mean that the usual way scientists use to work out what treatments work (the randomised controlled trial) is more or less impossible. Few rheumatologists will ever see more than a handful of Felty’s syndrome patients in their careers, so we do not have a strong evidence base for treatment decisions.

 

From the imperfect research literature we have, the most effective treatment is Methotrexate. This is the treatment of choice for rheumatoid arthritis, but it can also cause a low white cell count. In Felty’s syndrome, we advise giving much lower than usual doses of the drug, with careful monitoring and review. In around 50% of cases, the white cell count will return to normal, but it may take a few months for this to happen. For those whose condition is responsive to Methotrexate, it is vital not to stop it for long (for example when recovering from an infection or surgery) because the white cell count can crash down again very quickly without Methotrexate.

 

When Methotrexate does not work, there are several other options but these are best discussed with a specialist because decision-making is complicated and depends on the individual characteristics of the patient.


Are there lifestyle changes that can help the condition?

The main risk with Felty’s syndrome comes from infections. Therefore, it is important to adopt as healthy a lifestyle as possible to reduce the risk of picking up infections. That means taking regular exercise, not smoking, and maintaining a healthy weight. Having a nutritious diet and managing stress in a healthy way is also key. It is really important to make use of all available health protection strategies too, like getting an annual flu vaccination and having the Pneumovax (Pneumococcal pneumonia vaccination) once every 10 years.
 

For more guidance on treating Felty’s syndrome you may like to book an appointment with a leading rheumatologist such as Dr Gerald Coakley. Visit Dr Coakley’s Top Doctors profile today for more information.

By Dr Gerald Coakley
Rheumatology

Dr Gerald Coakley is a highly experienced consultant physician based in London. He has over 20 years’ experience in the diagnosis and management of fatigue syndromes including post-viral fatigue, post-Covid 19 syndrome (“Long Covid”) and myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS).

He ran a weekly fatigue clinic at Keats House, St Thomas Street from 2007 to 2023. Following the successful introduction of video consulting during the pandemic, and the publication by Penguin of his book Living with ME and Chronic Fatigue Syndrome, Dr Coakley is now concentrating on patients with ME/CFS and related conditions, offering exclusively video consultations. His experience is that video consultations work very well for fatigue disorders, and have opened up the option of medical advice for the severely affected group who are unable to leave their homes.

To make the most efficient use of time, he recommends that any patient seeking his opinion asks their primary care physician to organise the investigations recommended in the 2021 NICE guidelines for ME/CFS to exclude general medical causes of fatigue before booking an appointment. These are: FBC, ESR, CRP, U&E, LFT, TFT, calcium and phosphate, HbA1c, ferritin, CK, coeliac screen, and urinalysis.

In selected cases, further investigation may be required such as MRI scans, sleep studies or endocrine testing, in which case Dr Coakley can either advise on which tests to request from your primary care physician or organise them at The Harley Street Clinic, for those living near the capital. After more than three decades in rheumatology, Dr Coakley is no longer offering consultations for rheumatological conditions, either in person or by video.

Dr Coakley graduated in 1989 from the Royal London Hospital Medical School and went on from his training to be awarded with an Arthritis Research Campaign Fellowship. During this Fellowship he made discoveries relating to the immunology and genetics of rheumatoid arthritis, leading to several publications in international journals and the award of a PhD by the University of London in 2000. He has published on Felty’s syndrome (a rare complication of rheumatoid arthritis), septic arthritis and ME/CFS. If you have any enquiries before booking an appointment, please contact Dr Coakley’s PA Anne Hillman at [email protected]

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