Background/Purpose:

Henoch-Schönlein purpura is the most common small vessel vasculitis in childhood and has a classic presentation of palpable purpura. The disease is typically benign and self limited although some patients have gastrointestinal or renal complications requiring more aggressive therapy. Rarely, a subset of patients have persistent or recurrent musculoskeletal, gastrointestinal, and/or cutaneous symptoms without renal involvement despite use of supportive therapy, nonsteroidal anti-inflammatories, and glucocorticoids. There is a paucity of literature regarding therapy of such patients.  Some patients respond well to further immunosuppression.  We report a case series of three such patients.   

Methods:

A retrospective chart review of patients with a diagnosis of Henoch-Schönlein purpura followed in the pediatric rheumatology clinic at Saint Louis University from 1997-2014.  All patients met classification criteria for Henoch-Schönlein Purpura with the course of disease, treatment regime, and number of relapses were recorded.  Patients referred to rheumatology in our institution generally had musculoskeletal, cutaneous manifestations, and /or mild gastrointestinal or renal involvement.  Patients with renal or severe gastrointestinal disease are traditionally followed by those specialists.

Results:

From 1997-2014, 204 patients were seen at Cardinal Glennon Children’s Medical Center in all clinics with a diagnosis of Henoch-Schönlein purpura.  Twenty four patients meeting classification criteria were referred to the pediatric rheumatology clinic with musculoskeletal, gastrointestinal, and/or cutaneous manifestations.  Of those 24 patients, 18 had non-renal disease and 6 had coexisting renal disease.  Of those patients with non-renal disease, 15 had resolution of disease with supportive therapy, nonsteroidal anti-inflammatories, and/or one course of corticosteroids.  Three patients with non-renal disease failed to control their disease with supportive measures, nonsteroidal anti-inflammatories, and/or glucocorticoids, even after 6-14 months with repeat courses of glucocorticoids at 0.5-1.0 mg/kg/day.  These patients were started on azathioprine for 12-19 months which allowed successful prednisone tapering and amelioration of symptoms.  Two of the three patients had cutaneous and gastrointestinal symptoms without musculoskeletal disease with remission preserved off all medications with the use of azathioprine at 1 mg/kg/day and 1.4 mg/kg/day.  The third patient with cutaneous, musculoskeletal, and gastrointestinal disease was successfully tapered off his prednisone and is currently in remission on azathioprine alone at 2 mg/kg/day over the last 12 months.

Conclusion:

Azathioprine can be used successfully to treat glucocorticoid resistant non-renal Henoch-Schönlein purpura.  To our knowledge this is the first published report documenting this therapy.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/azathioprine-for-glucocorticoid-resistant-non-renal-henoch-schonlein-purpura/