Autoinflammatory service

The autoinflammatory service is one of a small handful in the UK. It manages patients with periodic fever syndromes as well as acquired autoinflammatory syndromes including VEXAS.

Autoinflammatory service referrals

These disorders present with episodes of sterile pyrexia, neutrophilic dermatoses, unexplained raised inflammatory markers and sepsis like episodes.

  • Familial Mediterranean fever (FMF)

  • Tumour necrosis factor receptor-associated periodic fever (TRAPS)

  • Cryopyrin-associated periodic syndromes (CAPS)

  • Familial cold autoinflammatory syndrome (FCAS)

  • Muckle-Wells syndrome (MWS)

  • NOMID/CINCAE

  • Schnitzler’s syndrome

  • PFAPA syndrome

  • Paraneoplastic Autoinflammation

  • VEXAS

  • Undifferentiated autoinflammatory syndromes- this makes up a significant proportion of patients seen.
  • Rigours

  • Myalgia

  • Synovitis

  • Serositis: peritonitis, pleuritis

  • Neutrophilic dermatoses

  • Lymphadenopathy

  • Oral and/or genital ulcer

  • Unexplained and severe recurrent pharyngitis

These disorders require a high degree of suspicion and hence the service is open to offer advice and guidance when appropriate. In VEXAS cases there is a national VEXAS MDT which may be an appropriate platform to discuss patients who may not be able to travel to our department for an initial appointment.

Important:

Please note

We will only accept referrals from secondary care after they have had thorough workup to exclude connective tissue disease, occult infection, and malignancy.

As the service is national and patients are required to attend a face-to-face appointment when first attending a thorough local work up is mandated before referral. This should include:

  • Investigations to exclude malignancy especially haematological e.g. CT scan, PET CT where appropriate, bone marrow biopsy if indicated.
  • Infection screen with a focus on excluding TB which may mimic autoinflammatory disorders.
  • Investigations and assessment to exclude connective tissue disease.
  • Serial CRPs
  • Baseline investigations that are required before starting Anakinra or Tocilizumab including a TB QuantiFERON, Hepatitis B and C screen, HIV screen and a chest X ray.

Routine Referral

Suspicion of a periodic fever syndrome/autoinflammatory disorder as described above.

Not to refer

  • Connective tissue disease- these should be referred to local rheumatology services.
  • Oral ulcers in isolation- such cases need to be referred to oral health.
  • Non-specific symptoms in the absence of recurrent fever and raised inflammatory markers.
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