OBJECTIVE: The study aimed to investigate the available clinical data regarding the most common early manifestations of urticaria and angioedema in conditions such as Sjögren syndrome, dermatomyositis, systemic scleroderma, and systemic lupus erythematosus (SLE). DATA SOURCES: A literature search was conducted of articles published between 2020 and 2024 using databases such as Scopus, Google Scholar, PubMed, and ScienceDirect, along with relevant sources published before 2020. STUDY SELECTION: One hundred two scientific papers were initially considered, with 45 remaining after full evaluation based on inclusion criteria such as publication date, country, journal, and availability. DATA EXTRACTION: Data were extracted by analyzing publication titles, abstracts, headings, introductions, main content, conclusions, and reference lists. DATA SYNTHESIS: The primary symptoms of urticaria and angioedema in connective tissue diseases were synthesized from evidence-based sources, emphasizing clinical cases across systemic lupus erythematosus, dermatomyositis, Sjögren syndrome, and systemic scleroderma. CONCLUSIONS: The prevalence of autoimmune angioedema in SLE ranged from 15% to 30%, with common areas affected being the face, lips, and periorbital region. In Sjögren syndrome, primary urticaria was observed in 26.2% of cases. The prevalence of edema and urticaria as primary symptoms in dermatomyositis remained uncertain, but localized edema of the face and limbs, as well as urticaria in areas like the neck and elbow creases, were noted. Systemic scleroderma often began with Raynaud phenomenon, accompanied by edema of the hands and fingers. Finally, expanding diagnostic criteria to include these symptoms as potential early indicators of autoimmune connective tissue diseases may help improve early detection and treatment strategies for these conditions.
Maria Z. Lisiecka (Mon,) studied this question.