SHM Converge Scientific Abstract ePoster Gallery-Case of Schnitzler Syndrome, Convergence of Rheumatology, Dermatology and Hematology

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This case describes a 61-year-old man with a 2-year history of migratory arthralgias, joint swelling, morning stiffness, fatigue, night sweats, fevers, weight loss, and a chronic non-pruritic urticarial rash. On exam, he had widespread urticaria on the trunk and lower extremities without lymphadenopathy or significant joint limitation.<br /><br />Workup showed elevated inflammatory markers (CRP 46 mg/L, ESR 39 mm/h) and an IgG lambda monoclonal gammopathy (M-protein 8.4 g/L). Ferritin was mildly elevated, while ANA, anti-dsDNA, RF, anti-CCP, infectious studies, and imaging were negative. Skin biopsy was consistent with urticaria. Abdominal ultrasound, chest X-ray, and PET scan did not reveal infection, inflammation, malignancy, or organomegaly.<br /><br />The differential diagnosis included lupus, hypocomplementemic urticarial vasculitis syndrome, angioedema, COPD, infection, adult-onset Still’s disease, and cryopyrin-associated periodic syndromes, but these were excluded based on clinical and laboratory findings. Schnitzler syndrome was considered most likely because of the combination of chronic urticaria, monoclonal gammopathy, recurrent fevers, and elevated CRP. The patient met Strasbourg diagnostic criteria, making the diagnosis definite.<br /><br />Treatment began with colchicine, which resolved the urticaria but not the systemic symptoms. After adding an IL-1 antagonist, his fevers, night sweats, and pain resolved within days, and inflammatory markers normalized over five months.<br /><br />The case highlights Schnitzler syndrome as a rare, often delayed diagnosis autoinflammatory disorder. IL-1 appears to play a central role in its pathophysiology, and IL-1 antagonists are now the preferred treatment, with tocilizumab as an alternative if needed.

Asset Subtitle

Babray Laek

Meta Tag

Author List Babray Laek, Jalil Makhkash, Mydah Hashmi, Truong Ngo, Zuhal Arzomand

Category Clinical Vignettes

Concept Schnitzler Syndrome

Concept Recurrent Fever

Concept Urticarial Rash

Concept Monoclonal Gammopathy

Concept Strasbourg Criteria

Distinguished Non-Finalist

Presenter Organization UPMC McKeesport

Presenting Author Babray Laek

Track Adult

Keywords

Schnitzler syndrome

chronic urticaria

monoclonal gammopathy

autoinflammatory disorder

IL-1 antagonist

fever

elevated CRP

arthralgia

Strasbourg criteria

tocilizumab

Schnitzler Syndrome

Recurrent Fever

Urticarial Rash

Monoclonal Gammopathy

Strasbourg Criteria