Table 1.
Author | Sex/Age (y) | Extra-Renal Organ Involvement | ANCA | Kidney Tests | Imaging | Localisation | Biopsy | Kidney Removal | Histology | Treatment | Outcome |
---|---|---|---|---|---|---|---|---|---|---|---|
Tiwari [10] | F/60 | ENT, eye | MPO | Normal | US, CECT, PET | Unilateral | Yes | No | Granuloma | MTX | Remission |
Maguire [11] | F/27 | ENT, lung | PR3 | NA | Renal arteriography | Unilateral | NA | Partial | Granuloma/Vasculitis | CYC | Remission |
Schapira [12] | M/45 | ENT, lung | NA | UP | Gallium scan, US | Unilateral | No | Partial | Granuloma/Vasculitis | CYC | Remission |
Schydlowsky [13] | M/47 | ENT, lung | Positive | Normal | US, CECT | Unilateral | No | Radical | Granuloma/Vasculitis | CYC | Remission |
Smith [14] | F/52 | Eye | NA | WBCs | US, CT | Unilateral | No | Radical | Granuloma/Vasculitis | AZA | Remission |
Boubenider [15] | F/45 | ENT, skin | PR3 | Renal failure, RBCs, UP | US, CT | Unilateral | Yes | Radical | Granuloma/Vasculitis | None | ESRD |
Fairbanks [16] | M/68 | ENT, lung | MPO | Normal | CECT | Unilateral | Yes | No | Granuloma/Vasculitis | MTX | Remission |
Dufour [17] | M/70 | ENT, PNS, lung | MPO | Renal failure | CT | Unilateral | No | Radical | Granuloma/Vasculitis | CYC | Relapsing disease |
Dufour [17] | M/67 | MSK, lung, CNS | PR3 | Renal failure | CT | Unilateral | NA | No | NA | CYC | Death |
Thomas [18] | F/48 | ENT, PNS, eye | NA | Renal failure | IVU, CT | Unilateral | Yes | Radical | Granuloma/Vasculitis | CYC | Remission |
Verswijvel [19] | M/24 | ENT, spleen | PR3 | Renal failure, RBCs | US, CECT, MRI | Unilateral | Yes | No | Vasculitis | CYC | Remission |
Carazo [20] | M/29 | Eye | PR3 | Normal | US, IVU, CECT | Bilateral | Yes | Radical | Granuloma/Vasculitis | CYC | Relapsing disease |
Kapoor [21] | M/22 | - | PR3 | Renal failure, RBCs, UP | US, MRI | Bilateral | Yes | No | Vasculitis | - | ESRD |
Leung [22] | M/66 | ENT, skin | MPO | Normal | US, CT | Bilateral | Yes | No | Granuloma | MTX | Remission |
D’Hauwe [23] | F/14 | ENT, MSK | Negative | WBCs | US, CECT | Unilateral | Yes | No | Granuloma | RTX+MTX | Remission |
Krambeck [24] | M/61 | ENT, CNS | Negative | Normal | CECT | Unilateral | No | Partial | Granuloma | AZA | Remission |
Vandergheynst [25] | M/32 | PNS | PR3 | UP | CT | Unilateral | Yes | Partial | Granuloma/Vasculitis | CYC | Remission |
Vandergheynst [26] | F/23 | ENT, skin, endocrine system | MPO | Normal | PET, CEUS | Bilateral | Yes | No | Granuloma/Vasculitis | RTX | Remission |
Sichani [27] | F/22 | ENT, lung, DAH | Positive | UP, RBCs | US, CECT | Unilateral | Yes | No | Granuloma/Vasculitis | - | Death |
Negi [28] | M/40 | ENT | PR3 | Renal failure | CT | Bilateral | No | No | NA | NA | Remission |
Lo Gullo [29] | M/38 | ENT, lung | PR3 | Normal | US, CECT, PET | Unilateral | Yes | No | Granuloma | RTX | Remission |
Frigui [30] | F/59 | ENT, eye | PR3 | UP | US, CECT | Bilateral | Yes | No | Granuloma/Vasculitis | CYC | Remission |
Xu [31] | M/55 | - | PR3 | RBCs | CT | Unilateral | No | Radical | Vasculitis | NA | Remission |
Roussou [32] | F/72 | ENT | MPO | Normal | CECT, MRI | Unilateral | No | Radical | Granuloma/Vasculitis | CYC | Remission |
Ahmed [33] | F/28 | Lung | PR3 | RBCs | US, CT | Bilateral | Yes | No | Granuloma/Vasculitis | CYC | Remission |
Ward [34] | F/48 | ENT, CNS, lung | PR3 | NA | CT | Unilateral | Yes | Radical | Granuloma/Vasculitis | CYC+RTX | Remission |
Yamamoto [35] | M/60 | - | MPO | Normal | CECT, MRI | Unilateral | Yes | Radical | Granuloma/Vasculitis | - | Remission |
Fu [36] | F/33 | ENT | Negative | Normal | US, PET | Bilateral | Yes | No | Granuloma/Vasculitis | CYC | Remission |
Higashihara [37] | F/75 | - | Negative | Renal failure | CECT | Unilateral | No | Radical | Granuloma/Vasculitis | - | Remission |
Dai [38] | M/32 | ENT, lung | PR3 | Normal | MRI, PET | Unilateral | Yes | No | Granuloma | CYC | Remission |
Guo [39] | F/71 | Lung, eye | PR3 | Normal | CT | Bilateral | Yes | No | Granuloma | RTX | Remission |
Kumar [40] | F/27 | ENT | PR3 | Normal | US, CECT, IVU | Unilateral | Yes | No | Granuloma | RTX | Remission |
Reeders [41] | M/46 | Lung, skin, MSK | PR3 | Renal failure | CT | Unilateral | No | Radical | Granuloma/Vasculitis | RTX+CYC | Remission |
Villa-Forte [42] | M/45 | ENT, lung | NA | NA | NA | Unilateral | No | Radical | Granuloma/Vasculitis | CYC | ESRD |
Boncoraglio [43] | M/47 | ENT | PR3 | Renal failure, RBCs, UP | US, CT, MRI | Unilateral | No | Partial | Granuloma/Vasculitis | RTX | Remission |
Abudaff [44] | M/20 | ENT | PR3 | Renal failure, RBCs, UP | US, CT | Unilateral | Yes | No | Vasculitis | RTX | Remission |
Bicakcigil [45] | F/47 | ENT, breast, spleen | NA | NA | CT | Bilateral | Yes | No | NA | CYC | Remission |
Gregorini [46] | M/46 | ENT, CNS | PR3 | Normal | CT | Unilateral | Yes | No | Granuloma | RTX+CYC | Remission |
Kaikoi [47] | F/76 | ENT, eye | PR3 | Minimal UP | CECT | Unilateral | Yes | No | Granuloma/Vasculitis | CYC | Remission |
Nketiah Sarpong [48] | M/57 | Lung, DAH | PR3 | Renal failure, RBCs, UP | NA | Unilateral | Yes | No | Granuloma/Vasculitis | RTX | Remission |
Ramasamy [49] | F/39 | NA | Negative | Renal failure | NA | Unilateral | No | Radical | Granuloma/Vasculitis | NA | ESRD |
Varkala [50] | M/22 | Lung, DAH, heart, skin | PR3 | Renal failure, RBCs, WBCs, granular casts | CECT | Unilateral | Yes | No | Vasculitis | CYC | Remission |
Present case | F/49 | - | PR3 | Normal | US, CECT, PET-MR |
Bilateral | Yes | No | Granuloma | RTX | Remission |
ANCA: antineutrophil cytoplasmic antibodies; AZA: azathioprine; CECT: contrast enhanced computed tomography; CEUS: contrast-enhanced ultrasonography; CNS: central nervous system; CT: computer tomography; CYC: cyclophosphamide; DAH: diffuse alveolar haemorrhage; ENT: ear, nose, and throat; ESRD: end stage renal disease; IVU: intravenous urography; MPO: myeloperoxidase; MRI: magnetic resonance imaging; MTX: methotrexate; NA: not available; PET: positron-emission tomography; PNS: peripheral nervous system; PR3: proteinase 3; RBCs: red blood cells; RTX: rituximab; UP: urine protein; US: ultrasound; WBCs: white blood cells.