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A Proposed Classification of the Immunological Diseases

Figure 1

The Immunological Disease Continuum, with Examples

The monogenic “autoinflammatory” diseases may be exclusively determined by local tissue-specific factors. For rare monogenic “autoimmune” conditions, the disease localisation appears to be determined predominantly by the adaptive immune response. The clinical heterogeneity within the immunological diseases, both among patients and between populations, may reflect the variable expression of autoinflammatory and autoimmune factors in disease causation. For example, in humans, there is considerable genetic and molecular evidence for uveitis falling into all of the disease categories, with the exception of the rare monogenic autoimmune diseases. There is also considerable overlap between polygenic autoinflammatory diseases and MHC class 1–associated diseases, but to simplify classification, these are split up into different categories. This figure does not include all immunologically recognised diseases because of their large number.

Figure 1

doi: https://doi.org/10.1371/journal.pmed.0030297.g001