Granulomatosis with polyangiitis in Tunisia

Ghorbel, I. Ben; Belfeki, Nabil; Baouendi, N.; Salem, T. Ben; Houman, M.H.

doi:10.4081/reumatismo.2017.935

Cited by 8 publications

(9 citation statements)

References 26 publications

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“…Renal involvement was present in 83.3% of patients, revealed by renal insufficiency in 83.3% of cases, proteinuria in 72% of subjects and hematuria in 55 % of cases. These clinical signs have been reported by many authors [11][12][13].…”

Section: Clinical Features Compared In Aav Diseasessupporting

confidence: 74%

ANCA Vasculitis in Algeria

Gadiri¹,

Meriche²,

Alliouch-Kerboua³

et al. 2019

Madridge J Immunol

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Background: Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis comprises microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Major target antigens of ANCA associated with vasculitis are myeloperoxidase (MPO) and proteinase 3 (PR3). MPO-ANCA is related to MPA and EGPA, and PR3-ANCA is the marker antibody in GPA.Objective: We aim to report the clinical-immunological characteristics of 92 patients with positive ANCA vasculitis Patients and Methods: 92 patients (64 Female et 28 male), with ANCA vasculitis according to the Chapel Hill classification. ANCA was performed by indirect immunofluorescence, supplemented by immune dot to determine their specificity MPO/PR3. Results:The mean age of patients was 51 years, the diagnosis was: 14 cases of GPA, 21 cases of microscopic polyangiitis (MPA), 04 cases of EGPA, 53 subjects had signs of overlap between the GPA and MPA. The clinical picture was dominated by renal disease followed by lung disease and rheumatologic signs. Some patients had cardiac involvement. 71 patients had p-ANCA (77, 2%), of which 43 was anti-MPO specificity (46.7%), 21 patients had c-ANCA (22.8%), including 9 with a specific anti-PR3 (98%), 40 patients showed no 2 searched specificities (43.4%). Conclusion:ANCA vasculitis is rare, clinical and immunological spectrum is very heterogeneous. The demonstration of ANCA directed vis-a-vis PR3 and MPO specific as an aid in the diagnosis of systemic vasculitis

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Section: Clinical Features Compared In Aav Diseasessupporting

confidence: 74%

ANCA Vasculitis in Algeria

Gadiri¹,

Meriche²,

Alliouch-Kerboua³

et al. 2019

Madridge J Immunol

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“…ENT involvement was found in 65.2% of our GPA patients, which is less than what is found in previous studies reporting 72%-99% [1,5,[13][14][15][16]. A study from Tunisia has reported initial ENT involvement of 56% and 83% at follow up [17]. Nose and sinus are reported to be affected by GPA in 55-90% of patients, which is comparable to our finding of 56.5% [8,9,16,18].…”

Section: Discussionsupporting

confidence: 55%

Ear Nose and Throat (ENT) Manifestations in Granulomatosis with Polyangiitis in Patients from Saudi Arabia

Arfaj¹

2019

AJIM

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Granulomatosis with polyangiitis (GPA) frequently affects ear, nose and throat (ENT) at disease onset. Our aim was to report on our experience with the ENT manifestations in GPA patients, therapy and outcome. A retrospective study of GPA patients was performed who followed up in Rheumatology clinics at King Khalid University hospital, Riyadh during the period 1990-2016. Demographics, different ENT manifestations, laboratory and diagnostic features, therapy and outcome of GPA patients were retrieved from their medical charts. ENT involvement was observed in 15 (65.2%) of the 23 GPA cases identified. Otologic symptoms were noted in 3 (13.0%), nose and sinus symptoms in 13 (56.5%) and throat symptoms in 3 (13.0%) GPA patients. Epistaxis (39.1%) was the most prevalent nose and sinus symptom followed by sinusitis (30.4%), otitis media and hearing loss were the frequent otologic symptoms, oral ulcers and hoarseness of voice constituted frequent throat symptoms in GPA patients. Of the 15 ENT-GPA patients, 9 were males and 6 were females (male: female; 1.5: 1). Their mean age at disease onset was 33.8 ± 18.3 (range 11-57) years and mean duration of disease was 10.1 ± 5.9 (range 1-20) years. ANCA was positive in 93.3% ENT-GPA patients, 73.3% had c-ANCA and 20.0% had p-ANCA. Infections were noted in 33.3% ENT-GPA patients that included pneumonia, septicemia, esophageal candidiasis, bacterial meningitis and herpes zoster. All patients received oral prednisolone, 60.0% received intravenous cyclophosphamide, 20.0% refractory cases received rituximab doses, and the disease outcome was good. Comparison of ENT-GPA with non-ENT GPA cohort showed that 26.7% ENT-GPA patients had renal involvement compared to 87.5% non ENT-GPA patients (p = 0.009). Our study showed that the frequency of ENT symptoms in our GPA patients was less compared to other studies, and the disease outcome was good. Renal involvement was significantly less frequent in ENT-GPA cohort compared to non ENT-GPA cohort.

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“…In another retrospective study (95) of 30 GPA patients conducted in Tunisia, ENT involvement was common during follow-up (83% of patients) but was rarely recognized at symptom onset (56% of patients). The renal involvement was frequent (56%) and significantly associated with poor prognosis (95). The rate of relapses was 36% and the overall 2year survival was much lower (60%) than that reported for highincome countries.…”

Section: Impact Of Ethnicity In Aav Severity and Treatment Approachmentioning

confidence: 79%

Unmet needs in ANCA-associated vasculitis: Physicians’ and patients’ perspectives

Quartuccio

Treppo²,

Urso³

et al. 2023

Front. Immunol.

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In recent years, clinical research has increased significantly and therapies for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis have improved. However, there are still unanswered questions and unmet needs about AAV patients. The purpose of this review is to examine the frontiers of research related to emerging biomarkers eventually predicting relapse, and new therapeutic approaches, not to mention new quality of life assessment tools. Identifying predictors of relapse may help optimize therapeutic strategies, minimize disease recurrence, and reduce treatment-related side effects. In addition, it is important to recognize that patients may suffer long-term consequences of the disease and its treatment, which, although life-saving, is often associated with significant side effects. Our goal, therefore, is to highlight what has been achieved, the pitfalls, and what still needs to be done, comparing the views of physicians and patients.

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Granulomatosis with polyangiitis in Tunisia

Cited by 8 publications

References 26 publications

ANCA Vasculitis in Algeria

ANCA Vasculitis in Algeria

Ear Nose and Throat (ENT) Manifestations in Granulomatosis with Polyangiitis in Patients from Saudi Arabia

Unmet needs in ANCA-associated vasculitis: Physicians’ and patients’ perspectives

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