Abstract
Autoinflammatory diseases are a heterogeneous group of disorders characterized by abnormal activation of the innate immune system. They are an entity distinct from autoimmune disease and primary immunodeficiency disease, but at times patients may have overlapping immunologic and clinical features. They include monogenic and polygenic disorders, and molecular genetics can aid in making the diagnosis. This chapter will focus on the pathophysiology, clinical phenotype, diagnosis, and treatment of hereditary periodic fever syndromes and NOD2-associated diseases.
| Original language | English |
|---|---|
| Title of host publication | Rheumatology for Primary Care Providers |
| Subtitle of host publication | A Clinical Casebook |
| Publisher | Springer International Publishing |
| Pages | 293-310 |
| Number of pages | 18 |
| ISBN (Electronic) | 9783030806996 |
| ISBN (Print) | 9783030806989 |
| DOIs | |
| State | Published - Jan 1 2021 |
Keywords
- Autoinflammatory disease
- Inflammation
- NOD2
- NOD2-associated disease
- Periodic fever syndrome
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