Picken MM. The Interpretation of Congophilia in Tissue Biopsies: Caution Required. Am J Kidney Dis. 2018 Sep;72(3):315-317. [PubMed link]
Although Congo red stain remains at the forefront of testing in the diagnosis of amyloidosis, its interpretation may be challenging in certain instances involving genuine cases of amyloidosis that show weak congophilia, as well as rare cases of FGN that may be also weakly congophilic.
Alexander MP, Dasari S, Vrana JA, Riopel J, Valeri AM, Markowitz GS, Hever A, Bijol V, Larsen CP, Cornell LD, Fidler ME, Said SM, Sethi S, Herrera Hernandez LP, Grande JP, Erickson SB, Fervenza FC, Leung N, Kurtin PJ, Nasr SH. Congophilic Fibrillary Glomerulonephritis: A Case Series. Am J Kidney Dis. 2018 Sep;72(3):325-336. [PubMed link]
The congophilic properties of organized fibrillary deposits should not be solely relied on in differentiating fibrillary GN from renal amyloidosis. Mass spectrometry and DNAJB9 immunohistochemistry can be useful in making this distinction.
Xing G, Gillespie R, Bedri B, Quan A, Zhang P, Zhou XJ. Proliferative glomerulonephritis with monoclonal IgG deposits in children and young adults. Pediatr Nephrol. 2018 Sep;33(9):1531-1538. [PubMed link]
The authors descrive 5 cases with an age range of 10-19 years old. All five cases showed a membranoproliferative pattern with abundant mesangial and subendothelial monoclonal IgG3 deposits (3 κ and 2 λ light chain, respectively). Despite various immunosuppressive treatments, the disease appears slowly progressive.
Seifert ME, Yanik MV, Feig DI, Hauptfeld-Dolejsek V, Mroczek-Musulman EC, Kelly DR, Rosenblum F, Mannon RB. Subclinical inflammation phenotypes and long-term outcomes after pediatric kidney transplantation. Am J Transplant. 2018 Sep;18(9):2189-2199. [PubMed link]
The authors found that subclinical inflammation phenotypes were prevalent in pediatric kidney recipients without clinical dysfunction and were associated with increased acute rejection and allograft failure.
Srivastava A, Palsson R, Kaze AD, Chen ME, Palacios P, Sabbisetti V, Betensky RA, Steinman TI, Thadhani RI, McMahon GM, Stillman IE, Rennke HG, Waikar SS. The Prognostic Value of Histopathologic Lesions in Native Kidney Biopsy Specimens: Results from the Boston Kidney Biopsy Cohort Study. J Am Soc Nephrol. 2018 Aug;29(8):2213-2224. [PubMed link]
Lesions that were independently associated with progression: inflammation in nonfibrosed interstitium, moderate and severe versus minimal interstitial fibrosis/tubular atrophy, moderate and severe versus minimal global glomerulosclerosis, moderate and severe versus minimal arterial sclerosis, and moderate and severe versus minimal arteriolar sclerosis.
Lusco MA, Fogo AB. Renal biopsy lesions in primary glomerular diseases with deposits. Nephrol Dial Transplant. 2018 Aug 1;33(8):1290-1291. [PubMed link]
The classic approach to renal pathology has been one of morphology, based on light microscopy, immunofluorescence and electron microscopy, integrated with clinical findings. As our understanding of glomerular disease expands, we move toward pathogenesis as an approach to diagnosis.
Le Quintrec M, Lapeyraque AL, Lionet A, Sellier-Leclerc AL, Delmas Y, Baudouin V, Daugas E, Decramer S, Tricot L, Cailliez M, Dubot P, Servais A, Mourey-Epron C, Pourcine F, Loirat C, Frémeaux-Bacchi V, Fakhouri F. Patterns of Clinical Response to Eculizumab in Patients With C3 Glomerulopathy. Am J Kidney Dis. 2018 Jul;72(1):84-92. [PubMed link]
In the study, 26 patients were included. After eculizumab treatment (median duration, 14 months), 6 (23%) patients had a global clinical response; 6 (23%), a partial clinical response; and 14 (54%), no response.
Alvarado AS, Andeen NK, Brodsky S, Hinton A, Nadasdy T, Alpers CE, Blosser C, Najafian B, Rovin BH. Location of glomerular immune deposits, not codeposition of immunoglobulin G, influences definitive renal outcomes in immunoglobulin A nephropathy. Nephrol Dial Transplant. 2018 Jul 1;33(7):1168-1175. [PubMed link]
Total patients: 80; IF with only IgA (n = 55) and IgA + IgG (n = 25). Only the location of glomerular immune deposits in the peripheral capillary walls was associated with a significantly increased risk for end-stage renal disease, transplant, death and/or doubling of SCr.
Said SM, Cosio FG, Valeri AM, Leung N, Sethi S, Salameh H, Cornell LD, Fidler ME, Alexander MP, Fervenza FC, Drosou ME, Zhang D, D'Agati VD, Nasr SH. Proliferative glomerulonephritis with monoclonal immunoglobulin G deposits is associated with high rate of early recurrence in the allograft. Kidney Int. 2018 Jul;94(1):159-169. [PubMed link]
Allograft proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID) was found to be a recurrent disease in most (89%) patients. Median time from transplant to diagnostic biopsy was 5.5 months. Mesangial proliferative glomerulonephritis was the most common pattern on the diagnostic biopsy with 89% of cases showing immunoglobulin G3 subtype restriction. A detectable serum paraprotein was present in 20% of patients.
Ravindran A, Fervenza FC, Smith RJH, Sethi S. C3 glomerulopathy associated with monoclonal Ig is a distinct subtype. Kidney Int. 2018 Jul;94(1):178-186. [PubMed link]
Ninety-five patients with C3G were tested for monoclonal Ig (MIg) of which 36 were positive. Mean age at diagnosis was 60 years, and among patient 50 years and older, 65.1% had a MIg. Hematologic evaluation revealed monoclonal gammopathy of renal significance in 26 patients, multiple myeloma in five, smoldering multiple myeloma in two, and chronic lymphocytic leukemia, lymphoma, or type I cryoglobulin each in one patient.
Sethi S, Rajkumar SV, D'Agati VD. The Complexity and Heterogeneity of Monoclonal Immunoglobulin-Associated Renal Diseases. J Am Soc Nephrol. 2018 Jul;29(7):1810-1823. [PubMed link]
Conditions caused by direct tissue deposition of MIg include common disorders, such as cast nephropathy, amyloidosis, and MIg deposition diseases, as well as uncommon disorders, such as immunotactoid glomerulopathy, proliferative GN with MIg deposits, light-chain proximal tubulopathy, and the rare entities of crystal-storing histiocytosis and crystalglobulinemia. Indirect mechanisms of MIg-induced renal disease can cause C3 glomerulopathy or thrombotic microangiopathy without tissue MIg deposits. A review.
Sethi S. The Changing Spectrum of Heroin-Associated Kidney Disease. Clin J Am Soc Nephrol. 2018 Jul 6;13(7):975-976. [PubMed link]
The kidney diseases can involve the glomerulus, interstitium, and vessels, and they can have different clinical presentations. A short review and comment on other paper.