The Systemic Autoinflammatory Diseases Service is the only centre in the UK dedicated to the diagnosis and care of patients with fever syndromes and related disorders. 

It is based at the National Amyloidosis Centre within the Royal Free Hospital, with access to state-of-the-art clinical and research facilities and a team of highly qualified staff. 

The service provides:

  • a detailed diagnostic assessment
  • DNA testing and genetic counselling
  • shared care with local teams and regular follow-ups for adolescent and adult patients with systemic autoinflammatory disorders (fever syndromes)
  • recommendations for treatment and monitoring to assess response to treatment 
  • nationally funded treatment for different fever syndromes including CAPS, TRAPS, Colchicine resistant FMF, MKD, Schnitzler’s syndrome and interferonopathies
  • information and support to patients, their families and health providers

Contact information

Email: rf.amyloidosisadmin@nhs.net

Systemic autoinflammatory diseases, more commonly known as fever syndromes, are very rare diseases caused by over activity in the body’s immune system.

They can be genetic or develop without an inherited cause. 

People with these syndromes usually suffer from repeated episodes of fever and general unwellness, sometimes with other symptoms such as rashes, stomach aches and painful swollen joints. 

Each time such an episode occurs, the patient recovers, usually within a few days, without any clear cause being identified. 

Common symptoms include:

  • high fever (above 38°C, often with chills and sweats)
  • general feeling of unwellness, like that experienced during the flu
  • limb aching
  • rash
  • red sore eyes
  • mouth ulcers
  • abdominal or chest pain which can be very severe indeed
  • headaches
  • swollen glands
  • in some syndromes a gradual onset of deafness

In most cases, symptoms begin in early childhood, although some people only develop the symptoms as adults.

Patients can be ill for a long time before they are referred to us and a diagnosis is made, mainly because the signs and symptoms are not specific, and the syndromes are rare.

Many patients are referred after undergoing extensive investigations elsewhere. A definite diagnosis of a fever syndrome is made in up to half of the patients referred.

A diagnosis is usually made by looking at your symptoms, undertaking blood tests and genetic testing.

Treatment is now available for many types of inherited periodic fever syndromes and patients usually have a normal life expectancy.

We tailor our approach according to the type of fever suspected and your individual needs. For many patients, daily medication is necessary to manage the disease.

When a fever syndrome is diagnosed, you will have follow-up appointments with our doctors and specialist nurses who will recommend appropriate ongoing treatments. 

Most patients will need long-term surveillance. Where possible, our team provide this care for adult patients and teenagers at the Royal Free Hospital and children at Great Ormond Street Hospital for Children. 

If you live far away from our centre, treatment may be shared with your local hospital or other regional centres. In these cases, we can help by providing:

  • regular advice to patients and doctors involved in their treatment
  • regular reviews at the Royal Free Hospital or Great Ormond Street Hospital for Children
  • arrangements for hotel accommodation during visits

There are more than 35 different fever syndromes that have been identified. The major ones are:

  • Familial Mediterranean Fever: the most common inherited fever syndrome. It is treated with a drug called colchicine which is safe to take during pregnancy and breastfeeding.
  • TNF receptor associated periodic syndrome (TRAPS): the second commonest inherited fever syndrome. 
  • Cryopyrin Associated Periodic Syndrome (CAPS).
  • Mevalonate Kinase Deficiency (MKD): previously known as hyper-immunoglobulin D syndrome and periodic fever syndrome — HIDS.
  • Deficiency of adenosine deaminase 2 (DADA2).
  • PFAPA (periodic fever, aphthous stomatitis, pharyngitis, adenitis): a childhood syndrome.
  • Schnitzlers Syndrome: this affects older adults.
  • VEXAS syndrome: this affects older adults — usually men.

The service accepts referrals from physicians.

Referrals should be addressed to:

  • Professor Helen Lachmann
  • Dr Charalampia Papadopoulou, consultant paediatrician

Send referrals to:
National Amyloidosis Centre
Royal Free Hospital
Pond Street
London 
NW3 2QG

Email: rf.amyloidosisadmin@nhs.net

Please include the following details in the referral:

  • Patient contact details, including full address, email and telephone number(s). 
  • Name and full contact details of referring consultant and/or GP. 
  • Any recent biopsies including bone marrow (date and hospital where performed) and a copy of the final biopsy report.
  • Results of previous imaging (date and hospital where performed) and a copy of the final report.
  • Echocardiogram, DPD or other bone scans, cardiac MRI, CT or CT/PET scan results, skeletal survey.
  • Any implanted metal work or devices such as pacemakers or ICD and if they are CMR compatible.
  • If the patient has organ failure, details of dialysis or organ transplantation.
  • Relevant blood tests, in particular:
    • Serum immunoglobulins, serum and urine electrophoresis and immunofixation and serum free light chain assays.
    • Renal and liver function, quantification of proteinuria.
    • Cardiac biomarkers such as NT pro-BNP and troponin.
    • Previous bloods which assess chronic inflammation such as CRP, ESR and WBC if inflammatory disorders are suspected.