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Table 5 Clinical, histopathological, therapeutic, evolutional, therapeutic and laboratory data of 25 patients from Northeastern Brazil diagnosed with granulomatosis with polyangiitis (GPA) between 2005 and 2016

From: Granulomatosis with polyangiitis in Northeastern Brazil: study of 25 cases and review of the literature

Patient Sex 1stM → D
(months)
Clinical manifestations Current 24-h UPO ANCA Biopsy Therapy
1 F 6 nephritic syndrome, hemoptysis, CKD 1461 c-Anca + Tu n/a CS, CFM
2 F 1 Hemoptysis, nephritic syndrome, AKI, purpura, sinusitis, mastoiditis, acral necrosis 816 c-Anca + 1:80 n/a CS, CFM
3 F 3.5 sinusitis, scleritis, multiple mononeuritis, pulmonary nodule, polyneuropathy, CHF, nephritic syndrome; AKI 3760 c-Anca + Tu Pulmonary nodule: granulomatous vasculitis with OP CS, CFM, RTX
4 M 6 laryngitis, sinusitis, pulmonary nodule, hemoptysis, upper respiratory tract stenosis 150 c-Anca + 1:320 n/a CS, CFM, RTX
5 F 66 sinusitis, polyarthritis, purpura laryngitis, bronchial stenosis 130 c-Anca - Skin: LCV CS, CFM
6 F 0.5 sinusitis, CKD, alveolar hemorrhage, death n/a c-Anca + 1:640 n/a CS, CFM
7 F 84 sinusitis, polyarthritis, saddle nose deformity, pulmonary nodule 74 c-Anca - Nasal mucosa: granulomatous vasculitis CS
8 F 36 multiple mononeuritis, transverse myelitis, neurosensory deafness, sinusitis, uveitis, polyarthritis,, polyneuropathy, laryngitis, pulmonary nodule 150 c-Anca - n/a CS, CFM, RTX
9 F 216 sinusitis, saddle nose deformity nephritic syndrome, polyarthritis, dialytic AKI n/a c-Anca + 1:40 n/a CS
10 F 12 neurosensory deafness, polyarthritis, nephrotic syndrome, nephritic syndrome, epistaxis, dialytic CKD, alveolar hemorrhage, death 385 c-Anca + 1:160 n/a CS, AZA, RTX
11 M 120 polyarthritis, purpura; sinusitis, orbital pseudotumor, otitis media, pericardial effusion 64 c-Anca + Tu n/a CS, AZA, RTX
12 F 0.5 polyarthritis, nephritic syndrome; epistaxis, mastoiditis, scleritis, pulmonary nodule, facial nerve paralysis, CKD, mitral insufficiency 1680 c-Anca + 1:160 Bronchial lesion: granulomatous vasculitis CS, AZA
13 M 5 neurosensory deafness, polyneuropathy, polyarthritis, purpura, acral necrosis, nephritic syndrome; pulmonary nodule 150 c-Anca + 1:640 Sural nerve: granulomatous vasculitis CS, AZA
14 F 5 neurosensory deafness, sinusitis, nephritic syndrome, pulmonary nodule 1078 c-Anca + 1:40 Kidney: diffuse proliferative GN CS, CFM
15 F 192 scleritis, polyarthritis, epistaxis, orbital pseudotumor, sinusitis, hemoptysis, AKI, amaurosis 511 p-Anca + 1:640 Kidney: acute tubular necrosis CS, CFM
16 F 42 nodular scleritis, neurosensory deafness, polyarthritis, pulmonary nodule, third-degree AVB, cutaneous nodule 200 c-Anca + 1:640 Skin: Churg-Strauss granuloma; pulmonary nodule: granulomatous vasculitis with OP CS, CFM
17 F 120 Purpura, hemoptysis, cutaneous nodule, polyarthritis, CKD, orbital pseudotumor, 900 c-Anca + 1:320 Nasal mucosa: granulomatous vasculitis CS, CFM, AZA, RTX
18 F 0.5 purpura, pulmonary-renal syndrome, death n/a c-Anca + 1:320 Skin: LCV CS, IMGN
19 M 1 polyarthritis, nephrotic syndrome, pulmonary-renal syndrome, death 26,350 c-Anca + 1:80 Bronchial: granulomatous vasculitis CS, CFM, PLASMF
20 F 1 sinusitis, purpura, polyarthritis, nephritic syndrome 775 c-Anca + 1:640 Kidney: FSGS CS, CFM, RTX
21 F 120 hemoptysis, otitis media, sinusitis 140 c-Anca + 1:640 n/a CS, AZA, RTX
22 F 32 Raynaud phenomen, vertigo, polyneuropathy, purpura, sinusitis 150 c-Anca + 1:20 Skin: LCV CS, AZA
23 F 3 polyarthritis, hemoptysis, nephritic syndrome, AKI, nephrotic syndrome 4300 c-Anca + 1:160 Kidney: pauci-immune crescentic GN CS, RTX
24 F n/a sinusitis, anosmia, pulmonary fibrosis n/a p-Anca + 1:640 n/a CS, CFM
25 F 3 Scleritis, purpura, polyarthritis, sinusitis, Cushing’s syndrome n/a c-Anca + 1:80 Skin: LCV CS, MTX
  1. M male, F female, 1stM → D time in months from first manifestation of GPA to diagnosis, ANCA antineutrophil cytoplasmic antibodies, UPO urine protein output, CFM cyclophosphamide, RTX rituximab, AZA azathioprine, CS corticosteroids, AKI acute kidney injury, IMGN immunoglobulin, PLASM plasmapheresis, GN glomerulonephritis, CKD chronic kidney disease, AVB atrioventricular block, CHF congestive heart failure, OP organizing pneumonia, LCV leukocytoclastic vasculitis, Tu titer unavailable, FSGS focal segmental glomerulosclerosis