TY - JOUR
T1 - Four distinct clinical phenotypes of vasculitis affecting medium-sized arteries
AU - Shirai, T.
AU - Shirota, Yuko
AU - Fujii, H.
AU - Ishii, T.
AU - Harigae, H.
N1 - Funding Information:
000 The authors thank the staff of the Department of Hematology and Rheumatology, Tohoku University, for helpful discussions. This work was supported by JSPS KAKENHI [grant numbers 16H06642 and 18K16136] to TS and a research grant from Kanae Foundation and Nanbyo Medical Foundation to TS.
Funding Information:
This work was supported by JSPS KAKENHI [grant numbers 16H06642 and 18K16136] to TS and a research grant from Kanae Foundation and Nanbyo Medical Foundation to TS.
PY - 2019/7/4
Y1 - 2019/7/4
N2 - Objective: Within the spectrum of polyarteritis nodosa (PAN), cutaneous PAN (cPAN) is further classified into mild cPAN and severe cPAN which presents with ulcers, necrosis, or neuritis. As distinguishing between severe cPAN and systemic PAN can be difficult, this study evaluated the clinical characteristics of patients with necrotizing arteritis of medium-sized arteries. Methods: Forty-one patients diagnosed with necrotizing arteritis of medium-sized arteries between 2008 and 2017 at our institution were enrolled in this study. Clinical background, laboratory findings, treatments, and rates of relapse and death were evaluated. Results: Thirty-six patients were classified as having cPAN (mild, 15; ulcer, nine; neuritis, eight; both, four), and five cases manifested systemic vasculitis. Clinical characteristics of mild cPAN included female predominance (84.6%) and younger age (median 31 years); those of systemic PAN included older age (median 71 years) and higher levels of inflammatory markers. Severe cPAN manifested with intermediate phenotypes. The median doses of prednisolone used to treat mild cPAN, severe cPAN, and systemic PAN were 20.0, 40.0, and 40.0 mg/day, respectively. Immunosuppressants were used in 20.0% of mild cPAN, 90.5% of severe cPAN, and 80.0% of systemic PAN patients. Although the mortality rates were indistinguishable, the relapse rates of severe cPAN (ulcer type) were significantly higher than those of other types (88.9%). Conclusion: The clinical characteristics of mild cPAN, severe cPAN (ulcer type), severe cPAN (neuritis type), and systemic PAN were distinct from each other. In particular, patients with severe cPAN (ulcer type) had higher relapse rates, indicating the importance of combination therapy.
AB - Objective: Within the spectrum of polyarteritis nodosa (PAN), cutaneous PAN (cPAN) is further classified into mild cPAN and severe cPAN which presents with ulcers, necrosis, or neuritis. As distinguishing between severe cPAN and systemic PAN can be difficult, this study evaluated the clinical characteristics of patients with necrotizing arteritis of medium-sized arteries. Methods: Forty-one patients diagnosed with necrotizing arteritis of medium-sized arteries between 2008 and 2017 at our institution were enrolled in this study. Clinical background, laboratory findings, treatments, and rates of relapse and death were evaluated. Results: Thirty-six patients were classified as having cPAN (mild, 15; ulcer, nine; neuritis, eight; both, four), and five cases manifested systemic vasculitis. Clinical characteristics of mild cPAN included female predominance (84.6%) and younger age (median 31 years); those of systemic PAN included older age (median 71 years) and higher levels of inflammatory markers. Severe cPAN manifested with intermediate phenotypes. The median doses of prednisolone used to treat mild cPAN, severe cPAN, and systemic PAN were 20.0, 40.0, and 40.0 mg/day, respectively. Immunosuppressants were used in 20.0% of mild cPAN, 90.5% of severe cPAN, and 80.0% of systemic PAN patients. Although the mortality rates were indistinguishable, the relapse rates of severe cPAN (ulcer type) were significantly higher than those of other types (88.9%). Conclusion: The clinical characteristics of mild cPAN, severe cPAN (ulcer type), severe cPAN (neuritis type), and systemic PAN were distinct from each other. In particular, patients with severe cPAN (ulcer type) had higher relapse rates, indicating the importance of combination therapy.
UR - http://www.scopus.com/inward/record.url?scp=85060642355&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85060642355&partnerID=8YFLogxK
U2 - 10.1080/03009742.2018.1551965
DO - 10.1080/03009742.2018.1551965
M3 - Article
C2 - 30672367
AN - SCOPUS:85060642355
VL - 48
SP - 308
EP - 314
JO - Scandinavian Journal of Rheumatology
JF - Scandinavian Journal of Rheumatology
SN - 0300-9742
IS - 4
ER -