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Lofgren Syndrome — SCE Rheumatology MCQ

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HardConnective Tissue DiseaseLofgren SyndromeSCE Rheumatology

A 6-year-old with confirmed PFAPA has highly disruptive attacks every four weeks. Infection and cyclic neutropenia have been excluded. What abortive regimen defines an adequate corticosteroid-response trial in the CARRA plan?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: B — Prednisolone ≤2 mg/kg (maximum 60 mg), one or two doses; fever resolution within 24 hours

Explanation lettering: B = shown as A · E = shown as B · D = shown as C · A = shown as D · C = shown as E

E is correct. The CARRA PFAPA corticosteroid arm defines response as fever resolution within 24 hours after prednisone or prednisolone up to 2 mg/kg, maximum 60 mg, given as a single dose or two divided doses. This is abortive, not chronic suppressive, therapy. Repeated long tapers and monthly intravenous pulses create unnecessary cumulative toxicity. Families should know that corticosteroids can shorten an attack yet sometimes shorten the interval to the next one; frequent disruptive disease may prompt discussion of prophylactic or surgical pathways.

Reference: CARRA consensus treatment plans for PFAPA: https://pmc.ncbi.nlm.nih.gov/articles/PMC7157990/