| Concomitant cryoglobulinaemic vasculitis alongside ANCA-associated vasculitis: concurrence or coincidental individual occurrence |
Jeong Yeop Whang1, Jang Woo Ha2, Jihye Chung3, Yong-Beom Park3,4, Jaewoo Song5, Younhee Park5
, and Sang-Won Lee3,4
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1Department of Medicine, Yonsei University College of Medicine, Seoul, Korea
2Division of Rheumatology, Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin, Korea
3Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
4Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Korea
5Department of Laboratory Medicine, Yonsei University College of Medicine, Seoul, Korea |
Corresponding Author:
Younhee Park , Tel: +82-2-2228-2449, Fax: +82-2-2227-8353, Email: younheep@yuhs.ac
Sang-Won Lee , Tel: +82-2-2228-1987, Fax: +82-2-393-6884, Email: sangwonlee@yuhs.ac
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Received: March 22, 2025; Revised: May 10, 2025; Accepted: April 3, 2026. |
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| Abstract |
Background/Aims: Cryoglobulinaemic vasculitis (CryoVas) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) share the common characteristic of being small vessel vasculitides but exhibit significant differences in pathologic findings. This study investigated the incidence rate of cryoglobulinaemia and the concurrence of AAV and CryoVas in AAV patients without chronic viral hepatitis or haematologic malignancies.
Methods: Among the 324 patients diagnosed with AAV, 136 with available cryoglobulin test results were included. Their clinical data were reviewed, and clinical and laboratory data, including AAV-specific indices at diagnosis, were recorded. Repeated cryoglobulinaemia was defined as cryoglobulin positivity in sera on at least two occasions separated by an interval of ≥ 12 weeks. The classification criteria for CryoVas were applied to all 136 patients with AAV.
Results: The median age of the patients was 61.0 years (50.0−69.0), and 41.2% were male patients. Of the 136 patients, 75, 31, and 30 were diagnosed with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic GPA, respectively. Cryoglobulin was detected in sera of only two patients, yielding a detection rate of 1.5%. Both patients exhibited repeated cryoglobulinaemia and presented with red skin spots, constitutional symptoms, articular involvement, and vascular involvement; enabling their classification as having CryoVas alongside MPA. Additionally, one patient displayed peripheral neuropathy, reduced serum C4 levels, and rheumatoid factor positivity.
Conclusions: This study represents the first demonstration that the incidence rates of cryoglobulinaemia and CryoVas are 1.5% in patients with AAV without chronic viral hepatitis or haematologic malignancies. |
| Keywords:
Cryoglobulins ; Antibodies, antineutrophil cytoplasmic ; Cryoglobulinemia ; Vasculitis |
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