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Research ArticleOriginal Article
Open Access

Common clinical pattern of antineutrophil cytoplasmic antibody -associated vasculitis

An experience from a multicenter study in Saudi Arabia

Jumanah J. Alfuwayris, Amal M. Almulhim, Mohammed A. Almansour, Marzouq A. Albadi, Ibrahim A. Alhomood, Faisal Alblewi and Abdullah U. Althemery
Saudi Medical Journal May 2023, 44 (5) 486-491; DOI: https://doi.org/10.15537/smj.2023.44.5.20220818
Jumanah J. Alfuwayris
From the Department of Rheumatology (Alfuwayris), Ministry of the National Guard – Health Affairs, King Abdullah International Medical Research Center, from the Department of Rheumatology (Alfuwayris), King Saud bin Abdulaziz University for Health Sciences; from Department of Rheumatology, King Fahad Medical City (Almulhim, Almansour, Alhomood, Alblewi); from Rheumatology Division (Albadi), Security Forces Hospital, Riyadh; and from Department of Clinical Pharmacy (Althemery), College of Pharmacy, Prince Sattam bin Abdulaziz University, Al-Kharj, Kingdom of Saudi Arabia.
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Amal M. Almulhim
From the Department of Rheumatology (Alfuwayris), Ministry of the National Guard – Health Affairs, King Abdullah International Medical Research Center, from the Department of Rheumatology (Alfuwayris), King Saud bin Abdulaziz University for Health Sciences; from Department of Rheumatology, King Fahad Medical City (Almulhim, Almansour, Alhomood, Alblewi); from Rheumatology Division (Albadi), Security Forces Hospital, Riyadh; and from Department of Clinical Pharmacy (Althemery), College of Pharmacy, Prince Sattam bin Abdulaziz University, Al-Kharj, Kingdom of Saudi Arabia.
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Mohammed A. Almansour
From the Department of Rheumatology (Alfuwayris), Ministry of the National Guard – Health Affairs, King Abdullah International Medical Research Center, from the Department of Rheumatology (Alfuwayris), King Saud bin Abdulaziz University for Health Sciences; from Department of Rheumatology, King Fahad Medical City (Almulhim, Almansour, Alhomood, Alblewi); from Rheumatology Division (Albadi), Security Forces Hospital, Riyadh; and from Department of Clinical Pharmacy (Althemery), College of Pharmacy, Prince Sattam bin Abdulaziz University, Al-Kharj, Kingdom of Saudi Arabia.
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Marzouq A. Albadi
From the Department of Rheumatology (Alfuwayris), Ministry of the National Guard – Health Affairs, King Abdullah International Medical Research Center, from the Department of Rheumatology (Alfuwayris), King Saud bin Abdulaziz University for Health Sciences; from Department of Rheumatology, King Fahad Medical City (Almulhim, Almansour, Alhomood, Alblewi); from Rheumatology Division (Albadi), Security Forces Hospital, Riyadh; and from Department of Clinical Pharmacy (Althemery), College of Pharmacy, Prince Sattam bin Abdulaziz University, Al-Kharj, Kingdom of Saudi Arabia.
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Ibrahim A. Alhomood
From the Department of Rheumatology (Alfuwayris), Ministry of the National Guard – Health Affairs, King Abdullah International Medical Research Center, from the Department of Rheumatology (Alfuwayris), King Saud bin Abdulaziz University for Health Sciences; from Department of Rheumatology, King Fahad Medical City (Almulhim, Almansour, Alhomood, Alblewi); from Rheumatology Division (Albadi), Security Forces Hospital, Riyadh; and from Department of Clinical Pharmacy (Althemery), College of Pharmacy, Prince Sattam bin Abdulaziz University, Al-Kharj, Kingdom of Saudi Arabia.
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Faisal Alblewi
From the Department of Rheumatology (Alfuwayris), Ministry of the National Guard – Health Affairs, King Abdullah International Medical Research Center, from the Department of Rheumatology (Alfuwayris), King Saud bin Abdulaziz University for Health Sciences; from Department of Rheumatology, King Fahad Medical City (Almulhim, Almansour, Alhomood, Alblewi); from Rheumatology Division (Albadi), Security Forces Hospital, Riyadh; and from Department of Clinical Pharmacy (Althemery), College of Pharmacy, Prince Sattam bin Abdulaziz University, Al-Kharj, Kingdom of Saudi Arabia.
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Abdullah U. Althemery
From the Department of Rheumatology (Alfuwayris), Ministry of the National Guard – Health Affairs, King Abdullah International Medical Research Center, from the Department of Rheumatology (Alfuwayris), King Saud bin Abdulaziz University for Health Sciences; from Department of Rheumatology, King Fahad Medical City (Almulhim, Almansour, Alhomood, Alblewi); from Rheumatology Division (Albadi), Security Forces Hospital, Riyadh; and from Department of Clinical Pharmacy (Althemery), College of Pharmacy, Prince Sattam bin Abdulaziz University, Al-Kharj, Kingdom of Saudi Arabia.
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  • For correspondence: [email protected]
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    - Means for laboratory parameters. CRP: C-reactive protein, ESR: erythrocyte sedimentation rate, Hg: hemoglobin, RBCs: red blood cells, HPF: high power field, WBC: white blood cell, EGPA: eosinophilic granulomatosis with polyangiitis, GPA: granulomatosis with polyangiitis, MPA: microscopic polyangiitis. *CRP was not needed for 2 patients with GPA. $ESR was not needed for one patients with GPA, &Urine samples were not collected from 4 patients with GPA and 4 patients with EGPA.24h urine protein was collected from only 1 patient: 4.9 g/L.

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    Figure 2

    - Common signs and symptoms in patients with eosinophilic granulomatosis with polyangiitis (EGPA) and GPA. EGPA: eosinophilic granulomatosis with polyangiitis, GPA: granulomatosis with polyangiitis. §8 of 18 patients with adult-onset asthma and 4 of 18 with refractory asthma. ¥based on renal biopsy: (6) GPA patient with pauci-immune glomerulonephritis [4 of them with crescent], (1) GPA with rapid progressive glomerulonephritis, (1) GPA with pauci-immune and IgA deposit glomerulonephritis, (1) GPA with pauci-immune and focal segmental glomerulonephritis, (1) GPA with focal necrotizing proliferation glomerulonephritis with IgA deposits, (1) GPA with focal necrotizing glomerulonephritis, (2) GPA with focal segmental glomerulonephritis, (1) GPA with fibrous crescent, sclerosed glomerular tuft, and severe vascular changes. Renal biopsy was not performed in 2 GPA and 1 EGPA patients with renal manifestations of acute kidney injury, hematuria, or proteinuria.

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    Table 1

    - Frequencies for categorical paramters (variables).

    VariablesEGPAGPAP-valueTotalMPATotal
    n (%)n (%)
    Number of patients183351253
    Average age (years)40.17 (13.1)43.61 (14.0)0.3942.39 (13.6)53.50 (13.4)42.81 (13.7)
    Females11 (61.1)20 (60.6)0.2431 (60.8)2 (100)33 (62.3)
    ANCA type<0.0001
    MPO4 (22.2)4 (12.1)8 (15.7)2 (100)10 (18.9)
    Negative14 (77.8)1 (3.1)15 (29.4)0 (0)15 (28.3)
    PR30 (0)28 (84.9)28 (54.9)0 (0)28 (52.8)
    Systems
    Upper airway18 (100)27 (81.8)0.0845 (88.2)1 (50.0)46 (86.8)
    Pulmonary18 (100)24 (72.7)0.0242 (82.4)1 (50.0)43 (81.1)
    Musculoskeletal8 (44.4)17 (51.5)0.7725 (49.0)0 (0)25 (47.2)
    Constitutional5 (27.8)17 (51.5)0.1422 (43.1)1 (50.0)23 (43.4)
    Renal1 (5.6)16 (48.5)0.00217 (33.3)2 (100)19 (35.9)
    Neurology11 (61.1)3 (9.1)0.00114 (27.4)1 (50.0)15 (28.3)
    Cutaneous5 (27.8)5 (15.2)0.1610 (19.6)0 (0)10 (18.9)
    Cardiology5 (27.8)1 (3.1)0.026 (11.8)0 (0)6 (11.3)
    Gastroenterology3 (16.7)3 (9.1)0.656 (11.8)0 (0)6 (11.3)
    Ocular1 (5.6)4 (12.1)0.645 (9.8)0 (0)5 (9.4)

    EGPA: eosinophilic granulomatosis with polyangiitis, GPA: granulomatosis with polyangiitis, MPA: microscopic polyangiitis, ANCA: antineutrophil cytoplasmic antibody, MPO: myeloperoxidase, PR3: proteinase-3

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    Common clinical pattern of antineutrophil cytoplasmic antibody -associated vasculitis
    Jumanah J. Alfuwayris, Amal M. Almulhim, Mohammed A. Almansour, Marzouq A. Albadi, Ibrahim A. Alhomood, Faisal Alblewi, Abdullah U. Althemery
    Saudi Medical Journal May 2023, 44 (5) 486-491; DOI: 10.15537/smj.2023.44.5.20220818

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    Common clinical pattern of antineutrophil cytoplasmic antibody -associated vasculitis
    Jumanah J. Alfuwayris, Amal M. Almulhim, Mohammed A. Almansour, Marzouq A. Albadi, Ibrahim A. Alhomood, Faisal Alblewi, Abdullah U. Althemery
    Saudi Medical Journal May 2023, 44 (5) 486-491; DOI: 10.15537/smj.2023.44.5.20220818
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    Keywords

    • antineutrophil cytoplasmic antibody
    • AN CA-associated vasculitis
    • autoimmune disorder
    • Saudi Arabia
    • clinical manifestations

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