
CAPS with rash, fever, red eye and muscle pain almost every afternoon: Lachmann HJ, Arthritis Res Ter, 2009. Open
Contents
Cryopyrin (cryopyrin) associated periodic syndromes (CAPS)
Three diseases with similar clinical features. It is considered among the interleukin-1-associated, auto-inflammatory periodic fever diseases. In general, FCAS is least complicated, MWS somewhat more and NOMID / CINKA most severe (please read more below)
Familial cold autoinflammatory syndrome (FCAS)
Develops during the first year of life
- Symptoms:
- Cold from air conditioning or climate regularly causes systemic inflammatory reaction
- Fever
- Cold urticaria (approx. 7 hours after cold exposure)
- Conjunctival redness (in eyes)
- Significant joint pain
- Leukocytosis (white blood cells) rises to 30.000 (after about 10 hours)
- Episodes progress gradually after 24 hours
- Cold from air conditioning or climate regularly causes systemic inflammatory reaction
Muckle-Wells syndrome (MWS)
Symptoms like at FCAS (see above) + following
- Intermittent (fluctuating) fever
- Headache
- Urticaria (rash)
- Arthralgia or arthritis
- Progressive (increasing) hearing loss
- Eye infection (uveitt)

CAPS (Yu JR, 2011). Open. Department of Dermatology, University of California, San Francisco, Divisadero Street, San Francisco, CA
Neonatal Onset Multisystem Inflammatory Disorder (NOMID) = CINCA Syndrome (Chronic Infantile Neutrophilic Cutaneous Articucular Syndrome)
- Chronic meningitis (meningitis, not bacterial)
- paralysis
- Cartilage and skeletal deformities
- Large spleen
- From birth a serious condition. Symptoms like MWS + the following:
Criteria for diagnosis
Criteria have been created for the diagnosis of several specialist groups
Diagnostic Criteria (Kuemmerle-Deschner JB 2017)
- High CRP (required for diagnosis)
Plus at least 2 of 6 by:
- Urticaria-like rash
- Coldness or stress have triggered episodes
- Sensory hearing loss
- Musculoskeletal symptoms (pain or swelling in joints or muscular pain)
- Chronic aseptic meningitis (not bacteria or viruses)
- Skeletal abnormalities
Other suggested criteria for diagnosis and classification (Federci, S, PRINTO / Eurofever 2015)
Occurrence Scores
- Urticarial rash 25
- Neurosensory hearing loss 25
- Conjunctivitis 10
- Absence of exudative pharyngitis 25
- Absence of abdominal pain 15
TOTAL points at least 52
Sensitivity: 70%. Specificity: 95%
Classification criteria Eurofever / PRINO 2019 (Gattorno M, 2019)
Genetic showing NLRP3 genotype and least en of the following:
- Urticarial rash
- Red eye (conjunctivitis, episcleritis or uveitis)
- Neurosensory hearing loss
OR
Absence of genetically showing the NLRP3 genotype and at least two of the following:
- Urticarial rash
- Red eye (conjunctivitis, episcleritis or uveitis)
- Neurosensory hearing loss
Literature
Auto-inflammatory diseases (BINDEVEVSSYKDOMMER.no)