Table 2.
Case reports
| Articles | Cases | Biopsy result | Treatment |
| Garg et al13 | 40-year-old man Serum creatinine of 20.8 mg/dL Urine Pro:Cr ratio of 4.7 g/g positive PR3-ANCA and MPO-ANCA Low complement C3 and C4 High ESR and CRP |
Diffuse tubulointerstitial fibrosis with majority of glomeruli globally sclerosed | High-dose steroids tapered to prednisone 40 mg/day. CRRT followed by IHD. |
| McGrath et al2 | Two patients with renal failure out of a series of 30 patients 100% MPO-ANCA positive 50% with PR3-ANCA positive First patient had Serum creatinine 7.7mg/dL Second patient had Serum creatinine of 5.6 mg/dL. UA for both patients with haematuria and proteinuria |
One patient underwent renal biopsy which was suggestive of pauci-immune focal necrotising and crescentic GN. | Treated with immunosuppression (specific treatment not mentioned in the article) |
| Carrara et al15 | 34-year-old woman Serum creatinine of 4.2 mg/dL Urine Pro:Cr ratio of 2.4 g/g UA with haematuria and proteinuria. MPO-ANCA positive and PR3-ANCA negative. |
Crescentic glomeruli (30%–40%), mild interstitial fibrosis. Granular staining for IgG kappa and lambda light chains. Numerous immune type electron dense deposits in intramembranous and subepithelial locations. | Pulse methyl prednisolone (1 g daily x 3 days). PLEX: seven sessions. Rituximab infusions (Two doses of 1 g each) and IHD. |
| Liu et al17 | 48-year-old woman Serum creatinine of 3.17 mg/dL Urine Pro:Cr ratio of 2 g/g Urine sediment with dysmorphic RBCs PR3-ANCA positive Low C3 and C4 |
Glomeruli have crescents and segmental sclerosis. 80% interstitial fibrosis. EM with unusual deposits of granules and rare single fibrils. |
Methylprednisolone 50 mg intravenously/day (duration not mentioned) with 50 mg/day prednisone taper for 6 weeks |
| Carlson et al12 | Patient 1: 60-year-old male Serum creatinine of 1.6 mg/dL Urine sediment with dysmorphic RBCs Urine Pro:Cr ratio of 2.0 g/g |
Necrotising pauci-immune GN | Prednisone, intravenous cyclophosphamide |
| Patient 2: 49-year-old woman Serum creatinine of 7.31 g/dL Urine sediment with dysmorphic RBCs Urine Pro:Cr ratio of 4.9 g/g |
Focal segmental and global sclerosing glomerulopathy with cellular crescents | Prednisone, plasmapheresis, intravenous cyclophosphamide | |
| Patient 3: 63-year-old woman Serum creatinine of 3.25 mg/dL Urine Pro:Cr ratio of 0.7 g/g. |
Necrotising pauci-immune GN | Prednisone, oral cyclophosphamide. | |
| Patient 4: 43-year-old woman Serum creatinine of 14.2 mg/dL Anuric |
Necrotising pauci-immune GN. | No treatment. | |
| Collister et al16 | Patient 1: 53-year-old man Serum creatinine of 5.08 mg/dL UA with haematuria and proteinuria Urine Pro:Cr ratio of 1.65 g/g PLA2R: Negative |
Active focal crescentic and necrotising GN, diffuse glomerular capillary wall thickening with epimembranous spikes. | Cyclosporine and ACE inhibitor. |
| Patient 2: 35-year-old woman with haemoptysis, arthritis Serum creatinine of 1.7 mg/dL Urine Pro:Cr ratio of 5.24 g/g. |
Active segmental fibrinoid necrosis. Diffuse foot process effacement and subepithelial and intramembranous immune deposits on EM. | Prednisone 40 mg oral twice a day. Cyclophosphamide 1 g intravenously followed by 750 mg intravenously every month for 6 months. Maintenance with azathioprine which he did not tolerate. Enalapril 10 mg daily. |
|
| Patient 3: 34-year-old male with CAL-induced AAV being treated with immunosuppression developed new-onset proteinuria. Serum creatinine of 0.8 mg/dL Urine sediment with dysmorphic RBCs Urine Pro:Cr ratio of 3.98 g/g. |
Focal intervening spikes of new basement membrane material. EM showed three glomeruli with subepithelial immune complex deposits. | Azathioprine and Irbesartan 150 mg oral daily for secondary membranous nephropathy. Azathioprine replaced with oral cyclophosphamide 150 mg and prednisone 60 mg daily due to lack of response. Cyclophosphamide changed to tacrolimus 4 mg twice daily due to complications from cyclophosphamide. Immunosuppression continued for 2 years, stopped 3 months after complete remission, prednisone taper continued for 9 more months. |
AAV, ANCA-associated vasculitis; CRP, C reactive protein; EM, electron microscopy; ESR, erythrocyte sedimentation rate; GN, glomerulonephritis; MPO, myeloperoxidase; PR3, proteinase 3; RBC, red blood cell; Urine Pro:Cr, Urine protein to creatinine ratio; ANCA, Anti neutrophil cytoplasmic antibodies; RRT, Renal replacement therapy; IHD, Intermittent Hemodialysis; UA, Urine analysis; PLEX, Plasma exchange; PLA2R, Anti Phospholipase A2 receptor antibody; CRRT, Continuous renal replacement therapy.