Review
Immunology
Nithya Rajendran, Puteri Maisarah Rameli, Hanaa Awad
Summary: This study aimed to investigate the risk factors for relapse in non-infectious cryoglobulinemic vasculitis (NICV). Through a systematic literature search and analysis, the study found that the subtype of cryoglobulinemic vasculitis, treatment regimens, and clinical and immunological responses were all associated with the risk of relapse. Appropriate risk stratification of NICV patients is crucial for successful treatment.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Immunology
Ming-Ling Chang, Jur-Shan Cheng, Ya-Hui Chuang, Li-Heng Pao, Ting-Shu Wu, Shiang-Chi Chen, Ming-Yu Chang, Rong-Nan Chien
Summary: This study investigates the evolution of cryoglobulinemia in Asian HCV-infected patients after sustained virological response (SVR) following direct-acting antiviral (DAA) treatment. The results show that cryoglobulinemia rates decrease in SVR patients over a 4-year follow-up period, with a shift towards single cryoglobulinemia. Improvements in cryoglobulinemia-associated markers are observed post-therapy, and baseline IgM levels can predict the occurrence of post-therapy cryoglobulinemia.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Medicine, General & Internal
Elena Treppo, Luca Quartuccio, Gaafar Ragab, Salvatore De Vita
Summary: HCV infection can lead to rheumatologic manifestations such as mixed cryoglobulinemia, which may progress to cryoglobulinemic vasculitis and non-Hodgkin lymphoma. Antiviral therapy is essential to eradicate HCV, while immunosuppressive therapy can be used for organ-threatening manifestations.
Review
Gastroenterology & Hepatology
Goncalo Boleto, Matheus Vieira, David Saadoun, Patrice Cacoub
Summary: CryoVas is a small-to-medium vessel systemic vasculitis caused by the deposition of mixed cryoglobulins and immune complexes, mainly triggered by HCV infection. The recent use of DAAs and B-cell depleting strategies, such as rituximab, has greatly improved the management of chronic HCV infection and HCV-related CryoVas. Despite advancements in recent years, there are still challenges in the therapeutic management of severe and refractory HCV-associated CryoVas.
CLINICS AND RESEARCH IN HEPATOLOGY AND GASTROENTEROLOGY
(2021)
Review
Virology
Cesare Mazzaro, Luigino Dal Maso, Laura Gragnani, Marcella Visentini, Francesco Saccardo, Davide Filippini, Pietro Andreone, Anna Linda Zignego, Valter Gattei, Giuseppe Monti, Massimo Galli, Luca Quartuccio
Summary: Chronic HBV infection can cause liver damage and lead to cryoglobulinemic vasculitis in some patients. Therapeutic options for HBV-related CV are currently lacking, with nucleos(t)ide analogues showing efficacy in controlling HBV replication and inducing clinical responses in mild-to-moderate CV patients, while plasma exchange and high-dose corticosteroids may be necessary in severe cases.
Article
Immunology
David Saadoun, Amine Ghembaza, Sebastien Riviere, Arsene Mekinian, Jonathan Boutemy, Gaelle Leroux, Fanny Domont, Helene Maillard, Mathieu Vautier, Patrice Cacoub
Summary: The combination therapy of belimumab and rituximab showed promising results in patients with refractory cryoglobulinemia vasculitis, leading to rapid clinical response.
JOURNAL OF AUTOIMMUNITY
(2021)
Article
Medicine, General & Internal
Arumugam Jeyapraniya, Shamila De Silva
Summary: The occurrence of autoimmune hepatitis in a patient with cryoglobulinemic vasculitis is extremely rare. We report a case of a 54-year-old woman with cryoglobulinemic vasculitis and autoimmune hepatitis.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Article
Medicine, General & Internal
Arumugam Jeyapraniya, Shamila De Silva
Summary: This case presents a rare occurrence of autoimmune hepatitis in a patient with cryoglobulinemic vasculitis, which is typically associated with hepatitis C.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Review
Gastroenterology & Hepatology
Anna L. Zignego, Silvia Marri, Laura Gragnani
Summary: Recent advances in the treatment of mixed cryoglobulinemia syndrome have shown significant progress, with interferon-free, DAA-based therapy leading to high SVR rates and clinical/immunological responses in most patients. However, challenges remain in managing partial or non-responders.
MINERVA GASTROENTEROLOGY
(2021)
Article
Immunology
Wei Bai, Lixia Zhang, Jiuliang Zhao, Shangzhu Zhang, Jiaxin Zhou, Xiaomei Leng, Zhengyin Liu, Wenling Ye, Bing Han, Xinping Tian, Mengtao Li, Yan Zhao, Xiaofeng Zeng
Summary: This study aimed to describe the main characteristics of 80 Chinese patients with cryoglobulinemia, finding that renal involvement and HBV infection may be more common. There were no significant differences in clinical or biological manifestations among patients with different causes of cryoglobulinemia.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Urology & Nephrology
Janina Paula T. Sy-Go, Charat Thongprayoon, Loren P. Herrera Hernandez, Ziad Zoghby, Nelson Leung, Sandhya Manohar
Summary: Patients with cryoglobulinemic vasculitis (CV) may experience disease flare after rituximab treatment, which tends to occur shortly after administration and affects the skin, kidneys, and peripheral nerves. It commonly leads to acute kidney injury and has a high mortality rate.
KIDNEY INTERNATIONAL REPORTS
(2021)
Review
Medicine, General & Internal
Mahmood Danishwar, Zahid Jamil, Salman Khan, Michael Nakhla, Ishtiaq Ahmad, Muhammad Ashar Ali, Daryl T. Y. Lau
Summary: Treatment with direct acting antivirals (DAAs) has greatly improved HCV therapy, but cryoglobulinemic vasculitis (CryoVas) can persist and recur after HCV cure. This review found that 63.7% to 90.2% of patients achieved a complete clinical response (CR) after DAA treatment, but 4% to 18% experienced relapse of CryoVas symptoms. Neuropathy, nephropathy, and dermatological complications were the most common manifestations of CryoVas, and B-cell clones persisted in a significant percentage of patients, potentially contributing to relapse. Certain gene variants were associated with a higher incidence of CryoVas persistence and recurrence.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Medicine, General & Internal
Jennifer Ngo, Robert Andalon, Luis Delgado, Mariam Gilmore, Kelly Downey, Patrick Wu, Made Sutjita
Summary: The effects of HCV on the nervous system primarily manifest through vasculitic processes in the peripheral nervous system. Although the causal relationship is still unclear, recent literature suggests an association between chronic HCV infection and transverse myelitis. This case report presents a rare instance of acute transverse myelitis occurring concurrently with a new diagnosis of HCV infection.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Review
Immunology
Clara Pouchelon, Marcella Visentini, Giacomo Emmi, Veronique le Guern, Luca Quartuccio, Maxime Samson, Nils Venhoff, Antoine Briantais, Milvia Casato, Emmanuel Chatelus, Marie Chilles, Maria C. Cid, Elisabeth Diot, Mikael Ebbo, Stanislas Faguer, Bernhard Hellmich, Marie Jachiet, Thomas Moulinet, Francois Perrin, Thomas Quemeneur, Renato Alberto Sinico, Benjamin Terrier
Summary: In patients with nonviral mixed CryoVas refractory to RTX, anti-CD20 plus belimumab, and alky-lating agents associated or not with anti-CD20, provide the highest rates of clinical response. However, anti-CD20 plus belimumab was frequently associated with severe infections.
AUTOIMMUNITY REVIEWS
(2022)
Article
Multidisciplinary Sciences
Batbold Batsaikhan, Ching-I. Huang, Ming-Lun Yeh, Chung-Feng Huang, Yi-Hung Lin, Po-Cheng Liang, Ming-Yen Hsieh, Yi-Ching Lin, Jee-Fu Huang, Wan-Long Chuang, Jin-Ching Lee, Ming-Lung Yu, Hsing-Tao Kuo, Chia-Yen Dai
Summary: This study found that the prevalence of persistent cryoglobulinemia is 34.5% after completing antiviral therapy. Persistent cryoglobulinemia is associated with advanced fibrosis and HCV clearance.
