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Intravenous Endotoxin Challenge in Healthy Humans: An Experimental Platform to Investigate and Modulate Systemic Inflammation

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[Systemic Inflammatory Response in a Young Woman].

Liubov Opokina¹, Philippe Rafeiner¹, Pia Schiffer¹

¹Klinik für Innere Medizin, Spital Zofingen, Privatklinik Villa im Park, Zofingen.

|January 5, 2022

Summary

A rare autoinflammatory condition, adult Still disease, was diagnosed in a young woman presenting with fever and rash. Treatment with tocilizumab effectively managed her systemic inflammatory response symptoms.

Keywords:

Adult Still disease Hautexanthem IL-6 Inhibitor IL-6 inhibitor Morbus Still Tocilizumab rare autoinflammatory disease seltene autoinflammatorische Erkrankung skin rash tocilizumab

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Area of Science:

Rheumatology
Immunology
Internal Medicine

Background:

Adult Still disease is a rare systemic autoinflammatory disorder characterized by fever, rash, and joint pain.
Diagnosis requires excluding other potential causes of systemic inflammation.

Observation:

A 21-year-old female presented with recurrent fever, arthralgias, and a characteristic salmon-colored rash.
Laboratory findings included neutrophilic leukocytosis, elevated C-reactive protein (CRP), ferritin, ASAT, and ALAT.

Findings:

The patient was diagnosed with adult Still disease after a thorough exclusion of differential diagnoses.
Initial treatment with non-steroidal anti-inflammatory drugs (NSAIDs) and prednisolone provided insufficient symptom control.
Escalation of therapy to tocilizumab, an interleukin-6 (IL-6) antagonist, resulted in significant symptom improvement.

Implications:

This case highlights the diagnostic challenges of adult Still disease.
It underscores the efficacy of IL-6 inhibition with tocilizumab in managing refractory adult Still disease.
Early recognition and targeted immunosuppressive therapy are crucial for favorable outcomes in patients with this rare condition.