44
Figure 6. Diagnosis of Autoinflammatory Syndromes
6.2 Evidence
of other PIDD,
malignancy, or
autoimmunity?
YES
6.1 Suspected
autoinflammatory
syndrome
6.3 Pursue
appropriate
diagnostic
evaluation
NO
6.4 Skin
pustules,
osteopenia, lytic
bone lesions
6.6 Arthritis,
skin ulcers,
and/or acne
6.8 Febrile
attacks with
rash, joint and
abdominal pain
6.10 Purpura,
lipodystrophy,
contractures,
muscle atrophy
6.5 Early-onset
rash with cold
exposure
6.7
Granulomas,
arthritis, uveitis,
rash
6.9 Bone lesions
and anemia
Test for IL-1RA
or IL-36RA
Test for
CIAS1 and
NLRP13, or
PLCG2
Test for
PSTPIP1
Test for NOD2
Test for FMF,
TRAPS, HIDS
Test for LPIN2
Test for PSMB8
Autoinflammatory Disorders
