Search

Type II cryoglobulinemia is a rare disorder characterized by abnormal immunoglobulins (Igs) precipitating in the blood at low temperatures and redissolving upon warming. Sjogren's disease (SjD) is an autoimmune disorder involving secretory gland malfunction that leads to persistent dryness of the mouth and eyes. Here, we report the case of a 61-year-old woman with a 7-year history of SjD who was diagnosed with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). However, her complicated clinical features could not be sufficiently explained by this disease alone. Immunofixation electrophoresis revealed monoclonal IgM-κ and polyclonal IgG-κ. The presence of precipitated cryoglobulin and elevated rheumatoid factor levels confirmed a diagnosis of type II cryoglobulinemia for this patient. To the best of our knowledge, this case represents the first report of a patient with CLL/SLL, SjD, and type II cryoglobulinemia, which increased our understanding of immune system-related disorders. Because certain similar mechanisms are involved in the pathogenesis of these three diseases, a combination treatment of rituximab, ibrutinib, and dexamethasone resulted in a favorable prognosis for this patient., Competing Interests: Declaration of conflicting interestThe authors declare that there is no conflict of interest.

Mixed cryoglobulinemia vasculitis (MCV) is caused in ~90% of cases by chronic hepatitis C virus (HCV pos MCV) and more rarely by hepatitis B virus (HBV) infection, or apparently noninfectious. HCV pos MCV develops in only ~5% of patients with chronic hepatitis C (CHC), but risk factors other than female gender have not been identified so far. We conducted a retrospective case control study investigating whether past active HBV infection, defined by hepatitis B surface antigen (HBsAg) seroclearance and anti-core antibody (HBcAb) positivity, could be a risk factor for developing HCV pos MCV. The prevalence of HBsAg seroclearance was 48% within 123 HCV pos MCV patients and 29% within 257 CHC patients (p=0.0003). Multiple logistic regression including as variables gender, birth year, age at HBV testing, cirrhosis, and hepatocellular carcinoma, confirmed an association of HBsAg seroclearance with HCV pos MCV [adjusted odds ratio (OR) 2.82, 95% confidence interval (95% CI) 1.73-4.59, p<0.0001]. Stratification by gender, however, showed that HBsAg seroclearance was associated with HCV pos MCV in male [OR 4.63, 95% CI 2.27-9.48, p<0.0001] and not in female patients [OR 1.85, 95% 95% CI 0.94-3.66, p=0.076]. HBsAg seroclearance, and more likely occult HBV infection, is an independent risk factor for HCV pos MCV in male CHC patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (Copyright © 2024 Morrone, Fiorilli, Cinti, Roberto, Ferri, Visentini, Pulsoni, Spinelli, De Santis, Antonelli, Basili, Tosti, Conti and Casato.)

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).)