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.
Hommos MS, Zeng C, Liu Z, Troost JP, Rosenberg AZ, Palmer M, Kremers WK, Cornell LD, Fervenza FC, Barisoni L, Rule AD. Global glomerulosclerosis with nephrotic syndrome; the clinical importance of age adjustment. Kidney Int. 2018 May;93(5):1175-1182. [PubMed link]
In patients with glomerulopathy that often presents with nephrotic syndrome, global glomerulosclerosis is clinically important only if it exceeds that expected for age.
Vazquez Martul E, Praga M. Nephropathology and nephrology. The need for a change. Nefrologia. 2018 May - Jun;38(3):247-249. [PubMed link]
A reflection on the integration of pathology and nephrology in medical centers.
Fukusumi Y, Zhang Y, Yamagishi R, Oda K, Watanabe T, Matsui K, Kawachi H. Nephrin-Binding Ephrin-B1 at the Slit Diaphragm Controls Podocyte Function through the JNK Pathway. J Am Soc Nephrol. 2018 May;29(5):1462-1474. [PubMed link]
Through interactions with nephrin, ephrin-B1 maintains the structure and barrier function of the slit diaphragm. Moreover, phosphorylation of ephrin-B1 and, consequently, JNK are involved in the development of podocyte injury. Another molecule more involved in the normal maintenance of the slit diaphragm.
Yeo SC, Cheung CK, Barratt J. Pediatr Nephrol. New insights into the pathogenesis of IgA nephropathy. 2018 May;33(5):763-777. [PubMed link]
Hasegawa J, Honda K, Omoto K, Wakai S, Shirakawa H, Okumi M, Ishida H, Fuchinoue S, Hattori M, Tanabe K. Clinical and Pathological Features of Plasma Cell-Rich Acute Rejection After Kidney Transplantation. Transplantation. 2018 May;102(5):853-859. [PubMed link]
The results indicated that plasma cell-rich acute rejection (PCAR) was an independent risk factor for allograft loss. PCAR presented with all types of rejection in the Banff 2015 criteria, and was associated with poor allograft survival.