McAdoo SP, Tanna A, Hrušková Z, Holm L, Weiner M, Arulkumaran N, Kang A, Satrapová V, Levy J, Ohlsson S, Tesar V, Segelmark M, Pusey CD. Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients. Kidney Int. 2017 Sep;92(3):693-702. [PubMed link].
No single-positive anti-GBM patients experienced disease relapse, whereas approximately half of surviving patients with AAV and double-positive patients had recurrent disease during a median follow-up of 4.8 years. Thus, double-positive patients have a truly hybrid disease phenotype, requiring aggressive early treatment for anti-GBM disease.
Beck LH Jr. PLA2R and THSD7A: Disparate Paths to the Same Disease? J Am Soc Nephrol. 2017 Sep;28(9):2579-2589. [PubMed link]
Oruc M, Durak H, Yalin SF, Seyahi N, Altıparmak MR, Trabulus S. A Rare Presentation of Immunoglobulin A Nephropathy: Acute Kidney Injury. Nephron. 2017;137(1):8-14. [PubMed link]
A total of 15 patients of 123 patients (12.2%) with primary IgAN admitted with AKI. On histology, cellular/fibrocellular crescents were present in 6 patients. In most cases (53.3%), pathologic abnormalities associated with acute tubular injury/necrosis were defined. Red blood cell casts in tubules were present in 6 cases (40%). In all cases, interstitial mixed inflammatory cell infiltration was observed.
Hilhorst M, van Paassen P, van Rie H, Bijnens N, Heerings-Rewinkel P, van Breda Vriesman P, Cohen Tervaert JW; Limburg Renal Registry. Complement in ANCA-associated glomerulonephritis. Nephrol Dial Transplant. 2017 Aug 1;32(8):1302-1313. [PubMed link]
C3c was found in 78 of 187 renal biopsies (41.7%). C4d was found positive in 70.8%, and properdin in 38.7%. In the majority of patients no immune complex deposits were found in their renal biopsies. The authors hypothesize that local immune complexes are quickly degraded and that complement activation occurs predominantly via alternative pathway.
Malvar A, Pirruccio P, Alberton V, Lococo B, Recalde C, Fazini B, Nagaraja H, Indrakanti D, Rovin BH. Histologic versus clinical remission in proliferative lupus nephritis. Nephrol Dial Transplant. 2017 Aug 1;32(8):1338-1344. [PubMed link]
One-third of patients who achieved a complete clinical response after induction had persistently high histologic activity, and 62% of patients who had complete histologic remission on rebiopsy were still clinically active. The kidney accrues chronic damage rapidly and despite clinical response in LN.
Roccatello D, Sciascia S, Rossi D, Alpa M, Naretto C, Radin M, Barreca A, Fenoglio R, Baldovino S, Menegatti E. High-Dose Rituximab Ineffective for Focal Segmental Glomerulosclerosis: A Long-Term Observation Study. Am J Nephrol. 2017 Jul 13;46(2):108-113. [PubMed link]
Only a minority (1 of 8) in our series of adult patients with FSGS showed positive effects of high doses of rituximab.
Arias LF, Taborada-Murillo A. Mesangial proliferative glomerulonephritis: A glomerular disease or a non-specific morphological change? Nephrology (Carlton). 2017 Jul;22(7):575. [PubMed link]
Isolated mesangial proliferation is a frequent and nonspecific change that should prompt a search for better characterized glomerulopathies. When no other morphological, immunopathological or ultrastructural alterations are found, the non-specific and controversial diagnosis of “mesangial proliferative glomerulonephritis” might be considered; however, this descriptive histological term does not aid in determining the aetiology or pathogenesis of a renal disease.
Park E, Ahn YH, Kang HG, Yoo KH, Won NH, Lee KB, Moon KC, Seong MW, Gwon TR, Park SS, Cheong HI. COQ6 Mutations in Children With Steroid-Resistant Focal Segmental Glomerulosclerosis and Sensorineural Hearing Loss. Am J Kidney Dis. 2017 Jul;70(1):139-144. [PubMed link]
In this report of 10 children with SR-FSGS and sensorineural hearing loss, the authors found 6 patients with biallelic COQ6 mutations. Median age at the onset of nephrotic syndrome was 29 months. All patients progressed to end-stage renal disease within a median of 13 months after the onset. Kidney biopsy revealed abnormal mitochondrial proliferation in podocytes in all 6 patients.
Malyszko J, Kozlowska K, Kozlowski L, Malyszko J. Nephrotoxicity of anticancer treatment. Nephrol Dial Transplant. 2017 Jun 1;32(6):924-936. [PubMed link]
Chemotherapeutic agents can affect the glomerulus, tubules, interstitium and renal microvasculature. The most nephrotoxic chemotherapeutic drug is cisplatin, which is often associated with acute kidney injury. A review.
Timmermans SAMEG, Abdul-Hamid MA, Vanderlocht J, Damoiseaux JGMC, Reutelingsperger CP, van Paassen P; Limburg Renal Registry. Patients with hypertension-associated thrombotic microangiopathy may present with complement abnormalities. Kidney Int. 2017 Jun;91(6):1420-1425. [PubMed link]
The authors evaluated the role of complement in nine patients with biopsy-proven renal TMA attributed to severe hypertension. In six out of nine patients, they found mutations C3 in three, CFI in one, CD46 in one, and/or CFH in two patients either with or without the risk CFH-H3 haplotype in four patients.
Luque Y, Louis K, Jouanneau C, Placier S, Esteve E, Bazin D, Rondeau E, Letavernier E, Wolfromm A, Gosset C, Boueilh A, Burbach M, Frère P, Verpont MC, Vandermeersch S, Langui D, Daudon M, Frochot V, Mesnard L. Vancomycin-Associated Cast Nephropathy. J Am Soc Nephrol. 2017 Jun;28(6):1723-1728. [PubMed link]
In renal tissue there is obstructive tubular casts composed of noncrystal nanospheric vancomycin aggregates entangled with uromodulin. The authors developed a new immunohistologic staining technique to detect vancomycin in renal tissue.