Pathology, Clinical Presentations, and Outcomes of C1q Nephropathy

Vizjak, Alenka; Ferluga, D; Rožič, Mojca; Hvala, Anastazija; Lindič, Jelka; Levart, Tanja Kersnik; Jurčić, Vesna; Jennette, J. Charles

doi:10.1681/asn.2007080929

Cited by 82 publications

(87 citation statements)

References 17 publications

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“…Electron-dense mesangial deposits were detected in 75-100% of C1q nephropathy cases in the native kidney 4,6,17 and in 82% of our cases. The absence of electron-dense deposits in a minority of cases of C1q nephropathy is likely due to sampling bias.…”

Section: Clinical Featuressupporting

confidence: 43%

“…The prevalence of C1q nephropathy among native kidney biopsies ranges from 0.2 to 1.9%. 2,4,6,10 The prevalence of intense C1q-dominant or codominant mesangial deposits among our renal allograft biopsies that underwent full IF analysis was 0.4%. This prevalence suggests that de novo 'C1q nephropathy' likely represents the third most common de novo morphological glomerular pattern after de novo focal segmental glomerulosclerosis (seen in 10-20% of allografts) and de novo membranous glomerulopathy (seen in 2-9% of allograft biopsies).…”

Section: Discussionmentioning

confidence: 89%

“…This recommendation is based on recent data indicating that proteinuria after kidney transplantation, even at low levels (o500 mg/day), is associated with reduced graft survival, independent of glomerular pathology, graft function, and acute rejection. 13,15 In the recent study by Vizjak et al, 6 of 72 cases of C1q nephropathy in the native kidney, 37.5% of patients had underlying infections, most commonly upper respiratory tract infection. Furthermore, the single previously reported patient with de novo 'C1q nephropathy' in the renal allograft had concurrent BK polyoma interstitial nephritis.…”

Section: Clinical Featuresmentioning

confidence: 98%

“…Others favor that C1q mesangial deposits reflects immune complex deposition. 6 It is possible that C1q, the first component of the complement cascade and an extremely basic immune reactant, binds to a polyanionic antigen present within the immune complexes deposited from the circulation or formed in situ. 2 The polyanionic antigen may be an intrinsic mesangial antigen or an extrinsic one (such as a viral nucleic acid or bacterial antigen).…”

Section: Clinical Featuresmentioning

confidence: 99%

“…4,5 More recent studies reported more heterogenous clinical presentations, histological findings, and outcomes of C1q nephropathy. Vizjak et al 6 reported 72 cases of C1q nephropathy from Slovenia, representing the largest cohort described to date. In their study, light microscopy revealed no lesions (38%), focal segmental glomerulosclerosis (15%), proliferative glomerulonephritis (28%), or various other lesions (19%).…”

mentioning

confidence: 99%

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C1q deposition in the renal allograft: a report of 24 cases

et al. 2010

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C1q nephropathy is an uncommon glomerular disease characterized by dominant or codominant mesangial staining for C1q in the absence of systemic lupus erythematosus. There are no series in the literature addressing the significance of C1q deposition in the renal allograft. We retrospectively analyzed 24 patients, most of whom were white (83%) and male (63%), with a mean age at transplant of 31 years. None of the patients were diagnosed with C1q nephropathy in the native kidney or had any features of systemic lupus erythematosus. The mean time from transplant to detection of mesangial C1q deposits was 37 months (412 months in 71% of cases). Half of the patients had a preceding infection. The indication for biopsy was surveillance (63%) or graft dysfunction (37%). At biopsy, 52% had proteinuria (41g/day in only 17%). The mean creatinine was 1.8 mg per 100 ml. Only 9% developed hematuria and none had hypoalbuminemia. The glomerular pattern on light microscopy was mesangial hypercellularity (46%), focal segmental glomerulosclerosis (21%), or no lesions (33%). All cases showed intense (Z2 þ ) dominant (67%) or codominant (33%) mesangial staining for C1q on immunofluorescence. Mesangial electron-dense deposits were seen in 82% of cases. On follow-up (mean 1 year) of the 10 patients without rejection, most had stable creatinine with no or stable proteinuria, and none lost their graft. We conclude that C1q-dominant mesangial deposition in the renal allograft is a morphological pattern with no apparent clinical significance in the majority of patients. It is usually detected after the first year. The rate of preceding infection and the prevalence of proteinuria seem to be similar to the renal transplant recipients in general. Most cases show mesangial hypercellularity or no glomerular changes on light microscopy. Modern Pathology (2010) 23, 1080-1088; doi:10.1038/modpathol.2010.92; published online 14 May 2010 Keywords: C1q nephropathy; transplant; de novo glomerular disease; FSGS; proteinuria; mesangial hypercellularityIn 1982, Jones and Magil 1 reported five patients with proteinuria and nonsystemic mesangiopathic glomerulonephritis, one of whom had C1q codominant mesangial deposits. In 1985, Jennette and Hipp 2 introduced the term 'C1q nephropathy' and defined it as dominant or codominant mesangial staining for C1q in patients lacking clinical or serological evidence of systemic lupus erythematosus. In their report, which included 15 patients who presented in their second or third decade of life with nephrotic-range proteinuria, 100% of patients had proteinuria (range 1-24 g/day), 40% hematuria, 0% hypocomplementemia, and 0% positive antinuclear antibodies. The light microscopic findings varied from normal-appearing glomeruli to mesangial hypercellularity to endocapillary hypercellularity. On immunofluorescence (IF), all cases showed intense segmental or global mesangial staining for C1q, which was accompanied by equal or weaker staining for C3 and immunoglobulins. On electron microscopy, all cases showed mesangial ele...

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Section: Clinical Featuressupporting

confidence: 43%

Section: Discussionmentioning

confidence: 89%

Section: Clinical Featuresmentioning

confidence: 98%

Section: Clinical Featuresmentioning

confidence: 99%

mentioning

confidence: 99%

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C1q deposition in the renal allograft: a report of 24 cases

et al. 2010

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Recombinant Spider Silk as Mediator for One‐Step, Chemical‐Free Surface Biofunctionalization

Horak

Jansson

Dev

et al. 2018

Adv Funct Materials

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A unique strategy for effective, versatile, and facile surface biofunctionalization employing a recombinant spider silk protein genetically functionalized with the antibody‐binding Z domain (Z‐4RepCT) is reported. It is demonstrated that Z‐silk can be applied to a variety of materials and platform designs as a truly one‐step and chemical‐free surface modification that site specifically captures antibodies while simultaneously reducing nonspecific adsorption. As a model surface, SiO2 is used to optimize and characterize Z‐silk performance compared to the Z domain immobilized by a standard silanization method. First, Z‐silk adsorption is investigated and verified its biofunctionality in a long‐term stability experiment. To assess the binding capacity and protein–protein interaction stability of Z‐silk, the coating is used to capture human antibodies in various assay formats. An eightfold higher binding capacity and 40‐fold lower detection limit are obtained in the immunofluorescence assay, and the complex stability of captured antibodies is shown to be improved by a factor of 20. Applicability of Z‐silk to functionalize microfluidic devices is demonstrated by antibody detection in an electrokinetic microcapillary biosensor. To test Z‐silk for biomarker applications, real‐time detection and quantification of human immunoglobulin G are performed in a plasma sample and C1q capture from human serum using an anti‐C1q antibody.

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Endocapillary proliferative glomerulonephritis associated with Q fever endocarditis: A case report

Coorey,

James,

Manickavasagam

et al. 2024

Clinical Case Reports

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Key Clinical MessageAcute Coxiella burnetti (Q fever) infection is known to activate the autoimmune inflammatory response. We report a rare case of glomerulonephritis associated with the Coxiella infection. An elderly male first presented with recurrent fevers of unknown origin and was subsequently diagnosed with Q fever infection and treated with doxycycline. He represented 2 months later with an acute kidney injury and active urinary sediment. Renal biopsy demonstrated a mesangiopathic pattern with staining for both C1q and IgM, raising possibilities of infection‐related glomerulonephritis, C1q nephropathy and lupus nephritis. This case demonstrates glomerulonephritis as a complication of Q fever infection and the differential diagnosis and workup up that needs to be considered in cases of chronic Q fever.

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Pathology, Clinical Presentations, and Outcomes of C1q Nephropathy

Cited by 82 publications

References 17 publications

C1q deposition in the renal allograft: a report of 24 cases

C1q deposition in the renal allograft: a report of 24 cases

Recombinant Spider Silk as Mediator for One‐Step, Chemical‐Free Surface Biofunctionalization

Endocapillary proliferative glomerulonephritis associated with Q fever endocarditis: A case report

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