Journal of Clinical Medicine Research, ISSN 1918-3003 print, 1918-3011 online, Open Access
Article copyright, the authors; Journal compilation copyright, J Clin Med Res and Elmer Press Inc
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Case Report

Volume 5, Number 4, August 2013, pages 316-321


Sustained Remission of Antineutrophil Cytoplasmic Antibody-Mediated Glomerulonephritis and Nephrotic Syndrome in Mixed Connective Tissue Disease

Figure

Figure 1.
Figure 1. Renal Biopsy findings: Light Microscopy (LM). Focal Proliferative and sclerosing glomerulonephritis with ten percent fibrous crescents, a) Glomerulus with fibro-cellular crescent and one globally sclerotic glomerulus - 200 ×; b) Globally and segmentally sclerosed glomeruli - 40 ×; c) Jones’ stain- glomerulus with fibro-cellular crescent - 200 ×; d) Glomerulus with fibro-cellular crescent and some segmental proliferation- 400 ×.

Tables

Table 1. Abnormal Serologies, Proteinuria and Serum Creatinine in the Patient of This Report
 
Laboratory valueNormal Range1st Period2nd Period3rd Period4th Period
1st Period: From the MCTD diagnosis until the development of the nephrotic syndrome (3 years); 2nd Period: Nephrotic syndrome prior to treatment (5 months); 3rd Period: Treatment with cyclophosphamide (1 year); 4th Period: Post-treatment follow-up (6 years). CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; SD: standard deviation; 95% CI: 95% confidence interval. Large confidence intervals with negative lower limits were computed in periods with two or three measurements.
Serum ANA titer< 1:40> 1:2,560> 1:2,560≥ 1:2,560≥ 1:2,560
Serum Anti-U1-RNP titer< 1:40> 1:512> 1:512> 1:512> 1:512
Serum MPO ANCA, u/L, Mean ± SD0 - 9935.1 ± 18.6789317.0 ± 306.3173.6 ± 91.8
95% CI-131.9-202.2-170.4-804.359.6 - 287.6
Serum C4 complement, mg/dL, Mean ± SD16 - 4710.5 ± 1.210.0 ± 1.211.4 ± 1.717.0 ± 1.7
95% CI7.4 - 13.58.9 - 11.110.1 - 12.715.5 - 18.4
Serum C3 complement, mg/dL, Mean ± SD88 - 20183.7 ± 9.1108.3 ± 15.8124.6 ± 12.6129.3 ± 10.6
95% CI60.9 - 106.493.6 - 122.9114.9 - 134.2120.3 - 138.2
Serum CRP, mg/dL, Mean ± SD0 - 11.5 ± 1.0-1.0 ± 0.60.4 ± 0.2
95% CI-0.9-3.920.1 - 2.00.1 - 0.8
ESR, mm/hr, Mean ± SD< 2084 ± 13-4024 ± 10
95% CI-30-1981 - 48
Serum creatinine, mg/dL, Mean ± SD0.66 - 1.250.75 ± 0.060.99 ± 0.161.00 ± 0.200.98 ± 0.06
95% CI0.66 - 0.840.85 - 1.120.82 - 1.180.93 - 1.02
Urine protein/creatinine, gr/gr, Mean ± SD< 0.11.2 ± 1.07.6 ± 2.36.4 ± 2.91.6 ± 1.0
95% CI-0.3-2.86.1 - 9.04.5 - 8.30.9 - 2.3

 

Table 2. Renal Manifestations, Treatment and Outcome in MCTD Patients With ANCA Positivity
 
ReferenceRenal Function and Urinary FindingsRenal HistologyTreatmentFollow-upOutcome
S: serum; NR: not reported; GN: glomerulonephritis; HPF: high power field; eGFR: glomerular filtration rate estimated by the modification in diet in renal disease (MDRD) formula.
8S. creatinine 1.5 mg/dL Proteinuria 0.77 g/day Hematuria > 100/HPFPauci-immune focal necrotizing GNMethyl-prednisolone, cyclophosphamide< 1 monthImproved proteinuria and serology
9S. creatinine NR Proteinuria NR Hematuria NRAtherosclerosis No GNPrednisolone Probucol Aspirin Sodium valproate1 monthImproved clinical picture and serology
10S. creatinine 4.08 mg/dL Proteinuria 6.5 g/day Hematuria NRFibrocellular crescents Pauci-immune necrotizing GNMethyl-prednisolone Cyclophosphamide Azathioprine14 monthsComplete Remission
11S. creatinine 6.0 mg/dL Proteinuria1+ Hematuria 3+Biopsy not doneMethyl-prednisolone Hemodiafiltration16 daysDied from pulmonary alveolar hemorrhage
12S. creatinine 4.0 mg/dL Proteinuria 2+ Hematuria PresentCrescentic pauci-immune GNMethyl-prednisolone Cyclophosphamide?/td>Improved renal function
13S. creatinine 2.49 mg/dL Proteinuria 1.7 g/day Hematuria PresentFibrocellular crescents Pauci-immune GN plus immune complexesPrednisolone116 daysDied from sepsis (colonic perforation)
14S. creatinine 0.5 mg/dL Proteinuria +/- Hematuria 0-1/HPFBiopsy not doneMethyl-prednisolone Azat hioprine42 daysImproved serology
This reportS. creatinine 0.9 mg/dL Proteinuria 11.0 g/day Hematuria 140/HPFFibrocellular crescents Pauci-immune GN plus immune complexesCyclophosphamide Prednisone6 yearsImproved clinical picture, proteinuria, serology, normal eGFR