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IgG4-related disease — SCE Rheumatology MCQ

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ModerateConnective tissue diseasesIgG4-related diseaseSCE Rheumatology

A 67-year-old man with biopsy-proven IgG4-related retroperitoneal fibrosis develops hydronephrosis. Examination shows flank tenderness is mild and blood pressure is controlled. Investigations show creatinine is rising and CRP is 36 mg/L. What is the most appropriate management?

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Correct answer: B — Start glucocorticoids and relieve urinary obstruction

Organ-threatening IgG4-related disease is treated with glucocorticoids while mechanical complications such as obstruction are managed urgently. Methotrexate alone would be too slow and does not address hydronephrosis. Before treatment, malignancy and infection should be reasonably excluded because IgG4-related disease can mimic both.

Reference: EULAR recommendations; BSR guideline; BNF