AUTOINFLAMMATORY VS AUTOIMMUNE

Autoinflammatory and Autoimmune Diseases Are Different

Mediated By

Autoinflammatory

Hyperactivation of the innate immune system¹

Autoimmune

Dysregulation of the adaptive immune system¹

Cell Production

Autoinflammatory

Dysregulated cytokine production²

Autoimmune

Production of autoantibodies³

Cell Involvement

Autoinflammatory

Myeloid cells⁴

Macrophages

Monocytes

Neutrophils

Autoimmune

Lymphoid cells⁴

T lymphocytes

B lymphocytes

Disease Examples

Autoinflammatory

(Example: SJIA)

Monogenic: FMF, CAPS, TRAPS, MKD, PAPA syndrome, Blau syndrome, and DIRA^5,6

Polygenic: SJIA, AOSD, PFAPA syndrome, and Behçet’s disease^5,6

Autoimmune

(Example: Rheumatoid arthritis)

Rheumatoid arthritis⁷
MS⁷
Systemic lupus erythematosus⁷

Select diseases and overlapping symptoms*

Autoinflammatory

(Example: SJIA)

Recurrent fever presenting at regular or irregular intervals or for ≥6 months^2,8
Rash^2,8
Arthritis²

Autoimmune

(Example: Rheumatoid arthritis)

Redness due to inflammation⁵
Arthritis lasting ≥6 weeks⁷

Overlapping symptoms can mean autoinflammatory diseases may be misdiagnosed as autoimmune diseases^5,6

Autoantibodies

Autoinflammatory

Negative autoantibody titers⁸

Autoimmune

Presence of autoantibodies specifically associated with autoimmune conditions⁸

Pathogenic Profiles

Autoinflammatory

Primarily driven by IL-1β, IL-6, and IL-18^8-10

Autoimmune

Primarily driven by IFN-γ and IL-17^10-12

*Symptoms vary based on specific disease.

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AOSD=adult-onset Still’s disease; CAPS=cryopyrin-associated periodic syndromes; DIRA=deficiency of the interleukin-1 receptor antagonist; FMF=familial Mediterranean fever; IFN-γ=interferon gamma; IL-1β=interleukin 1 beta; IL-6=interleukin 6; IL-17=interleukin 17; IL-18=interleukin 18; MKD=mevalonate kinase deficiency; MS=multiple sclerosis; PAPA=pyogenic arthritis, pyoderma gangrenosum, and acne; PFAPA=periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis; SJIA=systemic juvenile idiopathic arthritis; TRAPS=tumor necrosis factor receptor–associated periodic syndrome.

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