|
| | Clinical features | Kidney biopsy | Antibodies and C3, C4 |
| Histology | IF and EM findings |
|
| Charney et al. # A [5] | Raynaud’s phenomenon, pulmonary fibrosis, esophageal dysmotility, and TTP | DPGN with thrombotic microangiopathy | Trace IgG, IgM, and no C1q, C3, IgA, or fibrinogen.
No deposits on EM | Positive ANA with 1 : 640 titer and speckled pattern and lupus anticoagulant.
Negative anti-dsDNA and ANCA. Normal C3, C4 |
|
| Charney et al. # B [5] | ND | DPGN with necrotizing lesions, crescents, and thrombotic microangiopathy | 2+ IgM and fibrinogen.
Few scattered subepithelial deposits on EM | Positive ANA with 1 : 640 and homogeneous pattern and positive anti-dsDNA. Negative ANCA. Low C3 and C4 |
|
| Charney et al. # C [5] | Arthritis, leukopenia, coombs-positive hemolytic anemia, and serositis | DPGN with 40% sclerosis and 10% cellular and fibrous crescents | IgM (2+), IgA (1+), C1q (1+), and fibrinogen (1+). Scattered mesangial deposits on EM | Positive ANA with 1 : 640 titer and speckled pattern and anti-dsDNA, SS-A, and SS-B. Negative ANCA. Normal C3, C4 |
|
| Charney et al. # D [5] | Diagnosed with SLE, features are not described | DPGN and thrombotic microangiopathy | Mild staining for IgG, IgM, and C3 | Positive ANA with 1 : 140 titer, homogeneous pattern, and positive dsDNA. Negative ANCA and anticardiolipin. Low C3 and C4 |
|
| Charney et al. # E [5] | Arthritis | DPGN and thrombotic microangiopathy | Mild IgG and C3.
Rare mesangial and intramembranous deposits on EM | Positive ANA with 1 : 320 titer, homogenous pattern, and anti-dsDNA, anticardiolipin IgG. Negative ANCA. Low C3 and normal C4 |
|
| Hervier et al. # A [6] | Arthritis, hemolytic anemia, thrombocytopenia, sinusitis, cerebral ischemia, and intra-alveolar hemorrhage | Diffuse and global proliferative and crescentic GN | Endomembranous and mesangial IgG, IgM, and C3 deposits | Positive ANA with 1 : 5120 titers, homogenous pattern, anti-dsDNA, and MPO ANCA. Low C3 |
|
| Hervier et al. # B [6] | Discoid lupus, arthralgia, thrombocytopenia, and epistaxis | Proliferative crescentic GN, without mesangial proliferation or IgG deposits | | Positive ANA with 1 : 640 titer, speckled pattern, anti-dsDNA, and MPO ANCA |
|
| Hervier et al. # C [6] | Arthritis, serositis, thrombocytopenia, anemia, rhinitis, excavated pulmonary nodules, and leukocytoclastic vasculitis | Proliferative and necrotic GN | IgG and IgA deposits | Positive ANA with 1 : 2560 titer, homogenous pattern, anti-dsDNA, MPO ANCA, lupus anticoagulant, and anticardiolipin. Low C3 and C4 |
|
| Hervier et al. # D [6] | Discoid lupus, arthritis, pericarditis, thrombocytopenia, and intra-alveolar hemorrhage | ND | ND | Positive ANA with 1 : 160 titer and MPO ANCA. Negative dsDNA. Low C3 |
|
| Li et al. # A [4] | Rash, hemolytic anemia | DPGN with fibrocellular crescents | No immunofluorescence deposits. Scanty subepithelial electron dense deposits on EM | Positive ANA, 1 : 320 titer, and homogenous pattern. Negative anti-dsDNA, anti-PR-3, and anti-MPO antibody. Low C3 and C4 |
|
| Akhtar et al. # A [3] | Arthritis, alopecia, and AIHA | Diffuse mesangial cellularity, segmental cellular proliferation with necrosis. No crescents | 1+ focal staining for IgG and C3 | Positive ANA with 1 : 2560 titer, peripheral pattern, and IgM, IgG anticardiolipin. Negative anti-dsDNA, RF, and ANCA. Low C3 and C4 |
|
| Akhtar et al. # B [3] | Arthritis, Raynaud’s phenomenon, serositis, rash, and oral ulcers | Cellular proliferation of glomeruli with areas of necrosis. No crescents | 1+ focal staining in some mesangial areas.
Small mesangial deposits | Positive ANA with 1 : 2560 titer, homogenous pattern, anti-dsDNA, and SS-A/SS-B. Negative RF, ANCA. Low C4 |
|
| Marshall et al. # A [7] | Pancytopenia | Segmental necrotizing GN with cellular crescents | 2+ IgG and C3, negative IgM, IgA, and C1.
No subendothelial electron dense deposits | Positive ANA, 1 : 160, speckled pattern, anti-dsDNA, anti-histone antibody, and P-ANCA. Negative C-ANCA. Low C3 and normal C4 |
|
| Marshall et al. # B [7] | Serositis, sinusitis, and pulmonary infiltrates | Diffuse thickening of glomerular basement membranes, segmental necrotizing GN with cellular crescents | 3+ IgG, 2+ C3, and trace IgM. No subendothelial electron dense deposits | Positive ANA with 1 : 80 titer, P-ANCA, and MPO antibody. Negative C-ANCA, anti-dsDNA. Normal C3 and C4 |
|
| Fayaz et al. # A [2] | Arthritis, rash, photosensitivity, and leukopenia | Segmental and focal proliferative fibrinoid necrosis | No IF deposits.
No electron dense deposits | Positive ANA with 1 : 640 titer, homogeneous pattern, and anti-dsDNA. Negative ANCA, anti-MPO, and PR-3 antibodies. Low C3, normal C4 |
|
