1. Systemic Autoinflammatory Syndromes
- Author
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Catharina M. Mulders-Manders, Jeroen Van der Hilst, Jos W. M. van der Meer, and Anna Simon
- Subjects
Abdominal pain ,medicine.medical_specialty ,business.industry ,Amyloidosis ,Peritonitis ,Arthritis ,Familial Mediterranean fever ,medicine.disease ,Dermatology ,Rash ,Pericarditis ,Medicine ,Serum amyloid A ,medicine.symptom ,business - Abstract
Autoinflammatory diseases are characterized by recurrent episodes of inflammation, accompanied by a wide range of inflammatory symptoms, including fever, abdominal pain, skin rash, arthralgias, and myalgias. There can be frank signs of peritonitis, pericarditis, or arthritis. Many autoinflammatory diseases have a genetic background, and major progress has been made in identifying specific disease-inducing mutations. The common pathophysiological feature of autoinflammatory syndromes is the increased production of interleukin-1 (IL-1), making drugs targeting this cytokine the treatment of choice. The exception to this is familial Mediterranean fever, where anti–IL-1-therapy is only indicated when treatment with colchicine is ineffective. Adequate control of inflammation is necessary to prevent amyloid A (AA) amyloidosis.
- Published
- 2019
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