RESUMEN
Fibrillary glomerulonephritis (FGN) is a rare immune-mediated glomerular disease traditionally characterized by the presence of amyloid-like, randomly aligned, fibrillary deposits in the capillary wall, measuring approximately 20 nm in diameter and composed of polyclonal IgG. FGN is usually a primary disease with no pathognomonic clinical or laboratory findings. More than that, on light microscopic evaluation, it can receive various histological patterns, rendering its diagnosis indistinguishable. However, the identification by immunohistochemistry of a novel biomarker, DNA-J heat-shock protein family member B9 (DNAJB9), has created a new era in FGN diagnosis even in the absence of electron microscopy. Typically, most patients manifest various degrees of renal insufficiency, hypertension, microscopic hematuria, proteinuria, and occasionally frank nephrotic syndrome. The prognosis is usually severe and progression to end-stage kidney disease (ESKD) is the rule, given that no specific treatment is available until now, despite the fact that in small studies rituximab-based therapy seems to alleviate the severity and improve the disease progression. Herein, we report the case of a 63-year-old Caucasian man presenting with uncontrolled hypertension, headache, shortness of breath, and lower limb edema. Diagnostic evaluation revealed mild deterioration of kidney function, nephrotic range proteinuria, and faint IgGκ monoclonal bands in serum and urine immunofixation. After negative meticulous investigation for secondary nephrotic syndrome causes, the patient underwent a kidney biopsy. Biopsy sample showed two glomeruli with mesangial expansion and thickened glomerular basement membrane (GBM) on light microscopy, a pattern masquerading as membranous nephropathy stage III-IV, while IgG and C3 were 1-2+ on GBM and mesangium in immunofluorescence. Thickened GBM with fibrils on electron microscopy were found, while DNAJB9 in immunohistochemistry was positive, confirming FGN. Once diagnosis of FGN was made, a combination of steroids with rituximab was initiated while the patient was receiving the standard anti-hypertensive therapy, simultaneously with a sodium-glucose cotransporter-2 (SGLT2) inhibitor. The 12-month follow-up showed approximately 85% decrease in proteinuria alongside stabilization of kidney function and blood pressure normalization. Hence, in this article, we aim to highlight that DNAJB9-associated FGN may mimic membranous glomerulopathy stage III-IV on light microscopy, especially when a small kidney sample with extensive involvement by fibrils of GBM is examined. Moreover, we underscore the fact that ultramicroscopic examination is of crucial importance in the differential diagnosis of glomerular deposition diseases and that DNAJB9 identification on immunohistochemistry consists of a revolutionary and robust biomarker in FGN diagnosis.
RESUMEN
BACKGROUND: Sleep disorders are very common in patients with chronic kidney disease and they may not always subside after kidney transplantation. AIM AND METHODS: The aim of this cross-sectional study was to evaluate the self-reported quality of sleep, insomnia problems in particular, and examine the factors that disturb sleep of kidney transplant recipients (KTx: n=152) in comparison to age- and sex-matched patients on dialysis (HD: n=67) and participants with normal renal function (NOR: n=49), through the administration of the Athens Insomnia Scale (AIS) at least six months after transplantation. Clinical and laboratory data, as well as health-related quality of life, depression, anxiety, post-traumatic stress symptoms, and the presence of restless legs syndrome (RLS) and pruritus were investigated in relation to sleep problems. RESULTS: The highest mean AIS score was observed in the transplant patients (KTx: 4.6±13.3 vs. HD: 3.8±8.1 vs. NOR: 2.4±10.2); both KTx and HD patients had a lower quality of sleep compared to participants with normal renal function. Multiple linear regression analysis showed that the determinants of the total AIS score were the frequency of post-traumatic stress symptoms, depression, RLS, diastolic blood pressure, and pain (all p<0.0001). CONCLUSION: Although amelioration of renal function post-transplantation improves several aspects of quality of life, it does not seem to have a beneficial effect on self-reported sleep.