More than 30% of patients with RP have another associated systemic disease, including necrotizing vasculitis, autoimmune rheumatic disease (rheumatoid arthritis and others), hematologic disorders (especially myelodysplastic syndromes), endocrine disease (Graves' disease and others), inflammatory bowel disease, or another autoimmune disease (Table 1). Additionally, isolated cases have been described following ear piercing and intravenous substance abuse (6,7). How these associated conditions lead to RP is unknown. Tissue inflammation with release of sequestered connective tissue or cell membrane antigenic epitopes leading to an autoimmune response has been postulated.
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