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We report the case of a 53-year-old woman with Sjögren syndrome and cryoglobulinemia. The patient presented with nephrotic syndrome, hematuria, and reduced estimated glomerular filtration rate. The kidney biopsy revealed diffuse endocapillary proliferation and leukocyte exudation with focal intraluminal hyaline thrombi, prominent tubulointerstitial inflammation, and vasculitis. Diffuse granular mesangial and segmental to global capillary wall staining was observed on immunofluorescence with antisera to C3 and immunoglobulin M (IgM), with less intense staining indicative of IgG and κ and λ light chains. A biopsy diagnosis of Sjögren syndrome-related cryoglobulinemic membranoproliferative glomerulonephritis and vasculitis was rendered. Subsequent investigations revealed the presence of circulating type II cryoglobulins with cryocrit of 9%. Although rare, Sjögren syndrome is the most common cause of non-hepatitis C virus-related mixed cryoglobulinemia. We discuss the possible pathogenic mechanisms involved in the development of mixed cryoglobulinemia and its evolution to lymphoma, as best described in the setting of hepatitis C virus infection. Although the specific antigen involved is unknown, it is likely that the mixed cryoglobulinemia in Sjögren syndrome is triggered by the long-term B-cell stimulation, resulting in clonal proliferation of B cells. Additional chromosomal aberrations and cytokine milieu alterations, as seen in hepatitis C virus infection, may result in prolonged B-cell survival and progression to non-Hodgkin lymphoma.

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BackgroundEmerging evidence suggests older adults may experience subtle hemothoraces that progress over several days. Delayed progression and delayed development of traumatic hemothorax (dHTX) have not been well characterized. We hypothesized dHTX would be infrequent but associated with factors that may aid prediction.MethodsWe retrospectively reviewed adults aged ≥50 years diagnosed with dHTX after rib fractures at two level 1 trauma centers (March 2018 to September 2019). dHTX was defined as HTX discovered ≥48 hours after admission chest CT showed either no or ‘minimal/trace’ HTX. Two blinded, board-certified radiologists reviewed inpatient chest imaging and classified injury patterns according to Chest Wall Injury Society (CWIS) taxonomy. Descriptive analysis was performed for demographic and hospitalization characteristics.ResultsWe identified 14 patients with pooled dHTX rate of 1.3%. After initial chest CT negative for concerning hemothoraces, the patients did not undergo follow-up imaging until new symptoms (shortness of breath, chest pain) developed: eight (57%) were not diagnosed until after discharge from initial hospitalization (mean (range): 9 (2–20) days after discharge). Aspirin and/or anticoagulants were involved in fewer than half of cases (43%). According to CWIS taxonomy, all patients had a series of posterolateral fractures with at least one offset or displaced fracture, and an average of six consecutive rib fractures. All patients underwent tube thoracostomy and six patients (42%)—all aged <65—underwent operative interventions.DiscussionPreliminary data suggest older adults with rib fractures may be at risk of experiencing delayed progression of trace hemothoraces or a delayed presentation of hemothoraces. Asymptomatic progression or readmission to other services/hospitals likely occurs and true dHTX rates are likely higher. Our preliminary findings suggest a possible anatomic explanation for severe chest wall injury patterns’ association with dHTX. Further characterization and capturing the true incidence of dHTX first requires wider recognition of this complication.