Swiss Medical Weekly
SARS-CoV2-induced Schnitzler syndrome, inflammatory flare and successful treatment by anakinra during pregnancy: a case report
By Louise Weible, Britta Maurer, Nicola Andina and 1 others.
Abstract
Introduction
Schnitzler syndrome is a rare acquired autoinflammatory disease characterised by a chronic urticarial rash, recurrent fever, arthralgia or arthritis, bone pain, elevated inflammatory markers, and monoclonal gammopathy. The disease is driven by dysregulated inflammasome activation and excessive interleukin-1 (IL-1) production. Because of its rarity, data on the management of Schnitzler syndrome during pregnancy remain extremely limited. We report the case of a pregnant woman presenting with a SARS-CoV-2-induced inflammatory flare that was successfully treated with anakinra, highlighting both the diagnostic and therapeutic challenges of this uncommon condition in pregnancy.
Case presentation
A pregnant woman in her early 40s with previously diagnosed Schnitzler syndrome presented with an acute inflammatory flare triggered by SARS-CoV-2 infection. Clinical manifestations included urticaria and musculoskeletal symptoms consistent with disease reactivation, along with elevated inflammatory markers. The diagnosis of Schnitzler syndrome had previously been established based on clinical criteria, supported by the presence of a chronic urticarial rash and monoclonal gammopathy in accordance with the Strasbourg and Lipsker diagnostic criteria. Due to the severity of the symptoms and the central role of IL-1 in disease pathophysiology, treatment with anakinra, an IL-1 receptor antagonist, was initiated. The patient experienced rapid clinical improvement, with resolution of systemic symptoms and normalisation of inflammatory parameters. Treatment was well tolerated, with favourable maternal and foetal outcomes throughout the pregnancy.