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
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 |