Table 1.
Clinical characteristics
| Case | Sex | Age (yr) | Ethnicity | Baseline Cr (mg/dl) | Cr at biopsy (mg/dl) | GFR at diagnosis (ml/min per 1.73 m2) | Hematuria | Proteinuria at biopsy | Follow-up proteinuria | Albumin (g/dl) | Edema | Lung symptoms | Medical comorbidities | Antibodies | Other relevant labs | Treatment | Dialysis | Transplant | Cr at last follow-up (mg/dl) | GFR at follow-up (ml/min per 1.73 m2) | Follow-up duration (mo) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | F | 68 | White | 1.6 | 9 | 4 | 3+ | 4 g/24 h | NA | 2.5 | Yes | SOB | Rheumatoid arthritis, NSAID use | MPO | NA | Cyclophosphamide, prednisone complicated by aspergillus pneumonia | Yes | Yes | NA | NA | NA |
| 2 | F | 21 | White | NA | 15 | 3 | Anuric | Anuric | ESRD | NA | NA | DAH, acute respiratory failure | NA | Anti-GBM | NA | PLEX, prednisone, and cyclophosphamide | Yes | No | ESRD | ESRD | 47 |
| 3 | M | 65 | White | 0.9 | 2.2 | 30 | >100 RBCs/HPF | 2.89g/24h | ESRD | 3.7 | No | Hemoptysis | Raynaud syndrome | pANCA, MPO | ANA 1:80 | prednisone, MMF switched to cyclophosphamide, PLEX, then azathioprine and prednisone | Yes | Yes | ESRD (5.38) after transplant nephrectomy | ESRD | 98 |
| 4 | F | 64 | AA | 2 | 2.5 | 24 | Moderate, 20−29 RBCs/HPF | 1.6 g/24h | 1.1 g UPC | 2.2 | Yes | Hemoptysis, DAH | Diabetes, hep C cirrhosis, HCC | pANCA, MPO, PR3 | ANA 1:320, +anticardiolipin IgM, c3 77, c4 14 | Prednisone and cyclophosphamide then MMF | No | None | 4.33 Deceased | 12 | 13 |
| 5 | F | 62 | Asian | NA | 1.09 | 51 | NA | 16.9g/24h | NA | 0.6 | Yes | NA | Liver dysfunction | NA | NA | None | NA | NA | 1.14 | 49 | 79 |
| 6 | F | 61 | AA | 0.8 | 9 | 5 | Few RBCs | 8 g UPC | 1.6 g/ 24 h | 1.4 | Yes | Pulmonary nodule | HTN, CKD, heavy smoker | NA | NA | Prednisone and cyclophosphamide | No | No | 1.32 | 49 | 10 |
| 7 | F | 66 | AA | 1.3 | 2.9 | 20 | NA | 3.5 g UPC | NA | NA | NA | NA | Bladder cancer, MGUS | pANCA | NA | NA | NA | NA | NA | NA | NA |
| 8 | M | 70 | White | 1.3 | 2.35 | 28 | >20 RBCs/HPF | 2 g/24 h | 1.3 g UPC | 3.4 | No | Cough | Previous MN with crescents, MGUS, resected pancreatic neoplasm, metastatic sarcomatoid carcinoma, HTN, DM | pANCA, MPO, PR3 | Minute IgG K | Prednisone and cyclophosphamide | No | No | 1.99 Deceased | 33 | 14 |
| 9 | M | 79 | White | 1.5 | 3 | 21 | 21−30 RBCs/HPF | 1 g UPC | 0.2 g UPC | 4.5 | No | None | HTN, MGUS, diabetes | pANCA, MPO | None | Cyclophosphamide, prednisone, then MMF | No | No | 2.02 | 32 | 46 mo |
| 10 | M | 56 | AA | 1.07 | 1.07 | >60 | Large | 4.7 g/24 h | 0.7 g/ 24 h | 2.7 | Yes | None | Diabetes, HTN | NA | None | Cyclophosphamide | No | No | 0.83 | >60 | 49 mo |
| 11 | F | 42 | NA | 1 | 1.8 | NA | >60 RBCs/HPF | 1.8 g UPC | 0.2 g UPC | 1.7 | NA | NA | Prior MPO-ANCA cutaneous vasculitis, history of cocaine abuse, positive MRSA skin culture | MPO | Positive ANA and anti-SSB | Prednisone, MMF | NA | NA | 0.8 | >60 | 41 mo |
| 12 | M | 26 | White | NA | 18.6 | 3 | Large | 2.8 g/24 h | 0.07 g UPC | 3.6 | None | Hemoptysis | Obesity | Anti-GBM >8 | None | PLEX, steroids, cyclophosphamide | Yes | Yes | 2.01 | 39 | 38 mo |
| 13 | M | 34 | AA | 1.03 | 1.18 | 93 | 45 RBCs/HPF | 1.8 g UPC | 1.2 g UPC | 3.8 | NA | NA | HTN, obesity | pANCA, MPO | ANA 1:40 | Prednisone, rituximab | No | No | 0.98 | 113 | 28 mo |
| 14 | M | 41 | White | 1.8 | 10.9 | 5 | Large | 9.5 g UPC | ESRD | 2.5 | None | NA | Prior infection-related GN, i.v. drug use (including cocaine), hep C, endocarditis, seizure | pANCA, MPO | Positive anticardiolipin and anti-SSB | None | Yes | No | ESRD (10.2) | ESRD | 4 mo |
| 15 | F | 20 | AA | 0.83 | 3 | 24 | 3−5 RBCs/HPF | 24.8 g/24 h | 3.4 g UPC | 1.4 | Yes | SOB | Schizophrenia, bipolar disorder, Bell palsy, obesity | Anti-PLA2R antibody | Low C4 (17), normal C3 | Prednisone, cyclophosphamide | No | No | 1.51 | 52 | 17 mo |
AA, African American; ANA, anti−nuclear antibodies; ANCA, anti−neutrophil cytoplasmic antibodies; Cr, creatinine; C3, complement factor 3; C4, complement factor 4; CKD, chronic kidney disease; DAH, diffuse alveolar hemorrhage; DM, diabetes mellitus; ESRD, end-stage renal disease; F, female; GBM, glomerular basement membrane; GFR, glomerular filtration rate; GN, glomerulonephritis; HCC, hepatocellular carcinoma; hep C, hepatitis C; HPF, high-power field; HTN, hypertension; labs, laboratory investigations; M, male; MGUS, monoclonal gammopathy of undetermined significance; MMF, mycophenolate mofetil; MN, membranous nephropathy; MPO, myeloperoxidase; MRSA, methicillin-resistant Staphylococcus aureus; NA, not available; NSAIDs, nonsteroidal anti-inflammatory drugs; PLA2R, M-type phospholipase A2 receptor; PLEX, plasma exchange; PR3, proteinase 3; RBCs, red blood cells; SOB, shortness of breath; SSB, anti-Sjögren's syndrome type B; UPC, urine protein-to-creatinine ratio.