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. Author manuscript; available in PMC: 2014 Jun 1.
Published in final edited form as: Clin Immunol. 2013 Apr 9;147(3):155–174. doi: 10.1016/j.clim.2013.03.016

Table 3. Distinctive characteristics and clues to the diagnosis of the autoinflammatory diseases.

FMF PAPA

  • Serositis (recurrent peritonitis and pleuritis)

  • 1-3 days-length attacks

  • Response to colchicine

  • Pyoderma gangrenosum

  • Sterile pyogenic arthritis

  • Severe acne
HIDS Early-onset IBD

  • Triggered by immunization

  • Painfull bilateral cervical lymphadenopathy Onset in the first year of life

  • Onset in the first 3 months of age

  • Severe bloody diarrhea

  • Folliculitis
TRAPS DITRA

  • Fever attacks of longer duration (> 7 days)

  • Periorbital edema

  • Migratory myalgia and rash

  • Generalized pustular psoriasis

  • High-grade fever
CAPS/FCAS CAMPS

  • Triggered by cold

  • Fever attacks of short duration (6 to 24 hours)

  • Plaque or pustular psoriasis
CAPS/MWS PRAAS

  • Sensorineural hearing loss

  • High frequency of amyloidosis if untreated

  • Panniculitis

  • Lipodystrophy

  • Basal ganglia calcification
CAPS/NOMID PLAID

  • Continuous symptoms

  • Chronic aseptic meningitis

  • Typical facies: frontal bossing and saddle nose

  • Cold-induced urticaria

  • Recurrent sinopulmonary infection

  • Autoimmune manifestations

  • Positive ANA
PGA APLAID

  • Granulomatous inflammation

  • Uveitis, skin rash and chronic arthritis triad

  • • Vesicopustular rash

  • Mild immunodeficiency
DIRA HOIL-1 DEFICENCY

  • Generalized pustular skin rash

  • Osteomyelitis

  • Early-onset

  • Dramatic response to anakinra

  • Severe immunodeficiency

  • Cardiac amylopectinosis
Majeed syndrome

  • Early-onset pustular dermatosis

  • Osteomyelitis

  • Dyserytropoietic anemia

CAPS: cryopyrin asscoiated periodic syndrome; NOMID: neonatal-onset multisystem inflammatory disease; MWS: Muckle-Wells syndrome; FCAS: familial cold autoinflammatory syndrome; FMF: familial Mediterranean fever; HIDS: hyperimmunoglobulinemia D syndrome with periodic fever; TRAPS: TNF receptor associated periodic syndrome; PGA: pediatric granulomatous arthritis; DIRA: deficiency of interleukin 1 receptor antagonist; PAPA: pyogenic arthritis, pyoderma gangrenosum and acne syndrome; EO-IBD: early-onset inflammatory bowel disease; DITRA: deficiency of interleukin 36 receptor antagonist; CAMPS: CARD14 mediated psoriasis; PRAAS: proteasome associated autoinflammatory syndromes;PLAID: PLCγ2-associated antibody deficiency and immune dysregulation; APLAID: autoinflammation and PLCγ2-associated antibody deficiency and immune dysregulation;