Schnitzler syndrome

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Diagnostic criteria

Diagnostic criteria for Schnitzler syndrome, adapted from Lipsker et al.:

 

Urticarial skin rash, monoclonal IgM component and at least 2 of the following criteria:

· Fever

· Arthralgia or arthritis

· Bone pain

· Lymphadenopathy

· Hepatomegaly or splenomegaly

· Acute phase response (i.e. high ESR, high CRP and/or leukocytosis)

· Bone morphology changes

 

Remarks:

* other causes for the symptoms must be excluded, see differential diagnosis below

* if a monoclonal IgG is found (instead of IgM): variant type Schnitzler syndrome

 

 

Differential diagnosis

- Adult-onset Still’s disease

- Cryopyrin-associated periodic syndromes (FCAS, CINCA, MWS)

- POEMS syndrome                                                        

- Acquired C1 inhibitor deficiency                               

- Systemic Lupus Erythematosus                                 

- Hypocomplementic urticarial vasculitis                    

- Malignancies: Waldenström’s macroglobulinaemia, Lymphoma, Myeloma

- Infections