Glomerular diseases are a major cause of chronic kidney disease worldwide, yet epidemiological data from Eastern Europe, and Romania in particular, remain scarce. This study aimed to characterize the spectrum of biopsy-proven glomerulopathies in Romania through a multicenter national registry over a 10-year period. We retrospectively analyzed 4047 native kidney biopsies performed between 2014 and 2023 across four national nephrology reference centers. Patient demographics, clinical presentation, and histopathological diagnoses were collected and categorized into primary and secondary glomerular diseases, glomerulosclerosis, tubulointerstitial nephropathies, hereditary nephropathies, and vascular nephropathies. The mean patient age was 48 years, 54.8% were male, and 51.4% presented with nephrotic-range proteinuria. The most common primary glomerulopathies were membranous nephropathy (16.7%), immunoglobulin A nephropathy (15.6%), focal segmental glomerulosclerosis (8.8%), and membranoproliferative glomerulonephritis (10%). Among secondary glomerular diseases, lupus nephritis (9.3%), diabetic nephropathy (8.5%), and vasculitis (7.7%) were most frequent. Marked inter-center variability was observed, with a notably high prevalence of membranous nephropathy in Iași (31.1%). Over the study period, the incidence of focal segmental glomerulosclerosis increased while immunoglobulin A nephropathy declined. This study provides the first nationwide epidemiological assessment of biopsy-proven glomerular disease in Romania, revealing both similarities and distinctive differences compared to patterns reported in other European countries.

VEXAS syndrome is a recently identified autoinflammatory disorder caused by somatic mutations in the UBA1 gene, leading to systemic inflammation and hematologic abnormalities. While its renal involvement remains poorly understood, reported cases suggest a diverse spectrum of kidney pathology. We present a 69-year-old male with a history of systemic inflammation who developed nephrotic syndrome and worsening kidney function. His disease course included recurrent fevers, pulmonary infiltrates, cutaneous vasculitis, and hematologic abnormalities. A kidney biopsy revealed secondary amyloidosis, indicating chronic inflammation. Genetic testing confirmed a UBA1 mutation (c.121A > G, p.Met41Val), establishing the diagnosis of VEXAS syndrome. Despite treatment with corticosteroids, cyclosporine, and interleukin-1 blockade, the patient deteriorated and ultimately succumbed to septic shock. A systematic review of biopsy-confirmed renal involvement in VEXAS syndrome identified 23 cases, with interstitial nephritis as the most frequent histopathologic finding, followed by vasculitis, IgA nephropathy, minimal change disease, and amyloidosis. Treatment responses varied, with limited efficacy of immunosuppressive therapies. This case highlights the under-recognized renal manifestations of VEXAS syndrome, demonstrating its diagnostic challenges and the need for heightened clinical suspicion. Further research is required to define optimal management strategies and improve patient outcomes.

Objectives: A percutaneous native kidney biopsy remains the gold standard for diagnosing glomerular, tubulointerstitial and vascular kidney diseases. Although generally safe, it may cause major haemorrhagic complications. Using the French national registry, Kaczmarek et al. developed a simplified bleeding risk score (anaemia, female sex, heart failure, acute kidney injury; range: 0–5), achieving an AUC of 0.755 in native biopsies. We aimed to externally validate this score in a Romanian cohort and to assess whether additional clinically relevant predictors could improve discrimination for major bleeding. Methods: We conducted a retrospective cohort study of all consecutive adults undergoing an ultrasound-guided percutaneous native kidney biopsy at a tertiary nephrology centre in Romania between January 2008 and December 2024. The primary outcome was a composite major bleeding event: clinically significant haematoma or haemorrhage, blood transfusion, angiographic intervention or nephrectomy. The Kaczmarek score was validated using a receiver operating characteristic (ROC) analysis. Candidate predictors were assessed by multivariable logistic regression, and a simplified score was derived from the regression coefficients. Results: Among 3081 patients, 162 (5.3%) experienced major bleeding. The Kaczmarek score showed modest discrimination (AUC: 0.624, 95% CI: 0.585–0.663). In the multivariable analysis (n = 2506 complete cases), anaemia, heart failure, solid neoplasm and fibrinogen were independently associated with major bleeding. An eight-component score (anaemia, heart failure, solid neoplasm, and fibrinogen < 511 mg/dL; female sex, hypertension, liver disease, and eGFR < 30 mL/min/1.73 m²) achieved an AUC of 0.676 (95% CI: 0.631–0.717), outperforming the Kaczmarek score. Conclusions: The French score performed only modestly in our cohort. Adding comorbidities, kidney function and haemostatic parameters, particularly fibrinogen, improved the risk discrimination.

Plasma cell dyscrasias can have a heterogenous onset and, also, kidney involvement, associated with various clinical presentations. This mandates always considering plasma cell dyscrasias as a possible diagnosis when faced with a case with unusual progression. We report the case of a 44-year-old male with a sellar mass that was diagnosed first as an atypical pituitary adenoma, who later developed kidney involvement manifested as renal insufficiency and proteinuria, with a pathology report suggesting focal and segmental glomerulosclerosis. Within a year, he was diagnosed with multiple myeloma, prompting pathological reevaluation of the sellar mass and the change of diagnosis to solitary sellar plasmacytoma.