Acquired Fanconi syndrome in mixed cryoglobulinemia patients: a single-center case series.

Autor: Xu Q; Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, 100730, People's Republic of China.; 4 + 4 Medical Doctor Program, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, People's Republic of China., Chen G; Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, 100730, People's Republic of China. ChenGang@pumch.cn., Wen Y; Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, 100730, People's Republic of China., Shi X; Department of Traditional Chinese Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, 100730, People's Republic of China., Ye W; Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, 100730, People's Republic of China., Qin Y; Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, 100730, People's Republic of China., Li X; Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, 100730, People's Republic of China. lixmpumch@126.com.
Jazyk: angličtina
Zdroj: Clinical rheumatology [Clin Rheumatol] 2024 Jul; Vol. 43 (7), pp. 2329-2336. Date of Electronic Publication: 2024 May 10.
DOI: 10.1007/s10067-024-06997-2
Abstrakt: Purpose: Cryoglobulinemia is a pathological condition characterized by the presence of cryoglobulins in the blood, with cryoglobulinemic glomerulonephritis being the most frequent form of renal involvement. Fanconi syndrome presents as a generalized dysfunction of the proximal tubule, characterized by the presence of polyuria, phosphaturia, glycosuria, proteinuria, proximal renal tubular acidosis, and osteomalacia. We aimed to present five cases co-occurring with Fanconi syndrome and cryoglobulinemia.
Methods: We retrospectively summarized the cases of five patients with Fanconi syndrome and cryoglobulinemia at Peking Union Medical College Hospital from January 2012 to June 2022. The clinical features, diagnosis, treatment, and prognosis were systematically analyzed.
Results: All five patients exhibited typical features of Fanconi syndrome, and cryoglobulinemia was concurrently detected in all cases. These patients also exhibit positive anti-nuclear antibody spectrum and hyperglobulinemia, and IgM constitutes the predominant monoclonal component in cryoglobulins. In addition to supplemental treatment, timely immunosuppressive therapy may potentially benefit the long-term renal prognosis of patients with this condition.
Conclusion: Our findings highlight the rare co-occurrence of Fanconi syndrome and cryoglobulinemia in clinical practice. Despite the lack of causal evidence, the coexistence of Fanconi syndrome and tubulointerstitial injury is also noteworthy in patients with cryoglobulinemia, underscoring the importance of thorough evaluation and tailored management in patients presenting with overlapping renal manifestations. Key Points • Patients with mixed cryoglobulinemia can clinically present with tubulointerstitial injury, specifically manifesting as Fanconi syndrome. • In addition to typical symptoms of Fanconi syndrome, these patients also exhibit positive anti-nuclear antibody spectrum and hyperglobulinemia, while IgM constitutes the monoclonal component in cryoglobulins. • Timely immunosuppressive therapy may improve long-term renal prognosis in these patients.
(© 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)
Databáze: MEDLINE
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