Ear Nose and Throat (ENT) Manifestations in Granulomatosis with Polyangiitis in Patients from Saudi Arabia
American Journal of Internal Medicine
Volume 7, Issue 2, March 2019, Pages: 41-45
Received: Apr. 7, 2019;
Accepted: May 16, 2019;
Published: Jun. 4, 2019
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Abdurhman Saud Al Arfaj, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
Najma Khalil, College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia
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.
Abdurhman Saud Al Arfaj,
Ear Nose and Throat (ENT) Manifestations in Granulomatosis with Polyangiitis in Patients from Saudi Arabia, American Journal of Internal Medicine.
Vol. 7, No. 2,
2019, pp. 41-45.
Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: An analysis of 158 patients. Ann Intern Med. 116, 6, 1992, 488 98.
Jennette JC. Nomenclature and classification of vasculitis: Lessons learned from granulomatosis with polyangiitis (Wegener’s granulomatosis). Clin Exp Immunol. 164, Suppl 1, 2011, 7 10.
Bacon PA. The spectrum of Wegener's granulomatosis and disease relapse. N Engl J Med. 352, 4, 2005, 330-2.
Puechal X. Antineutrophil cytoplasmic antibody-associated vasculitides. Joint Bone Spine. 74, 5, 2007, 427-435.
Reinhold-Keller E, Beuge N, Latza U, et al. An interdisciplinary approach to the care of patients with Wegener’s Granulomatosis: long-term outcome in 155 patients. Arthritis Rheum. 43, 5, 2000, 1021-1032.
Laudien M, Hasler R, Wohlers Laudien M, et al. Molecular signatures of a disturbed nasal barrier function in the primary tissue of Wegener’s granulomatosis. Mucosal Immunol. 4, 5, 2011, 564-73.
Cadoni G, Prelajade D, Campobasso E, et al. Wegener’s granulomatosis: a challenging disease for otorhinolaryngologists. Acta Otolaryngol. 125, 10, 2005, 1105-10.
Gubbels SP, Barkhuizen A, Hwang PH. Head and neck manifestations of Wegener's granulomatosis. Otolaryngol Clin North Am. 36, 4, 2003, 685-705.
Srouji IA, Andrews P, Edwards C, Lund VJ. Patterns of presentation and diagnosis of patients with Wegener's granulomatosis: ENT aspects. J Laryngol Otol. 121, 7, 2007, 653-8.
Ponniah I, Shaheen A, Shankar KA, Kumaran MG. Wegener’s granulomatosis: the current understanding. OralSurg Oral Med Oral Pathol Oral Radiol Endod. 100, 3, 2005, 265-70.
Alam DS, Seth R, Sindwani R, Woodson EA, Rajasekaran K. Upper airway manifestations of granulomatosis with polyangiitis. Cleve Clin J Med. 79 Suppl, 3, 2012, S16-21.
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 65, 1, 2013, 1 11.
Kühn D, Hospowsky C, Both M, Hey M, Laudien M. Manifestation of granulomatosis with polyangiitis in head and neck. Clin Exp Rheumatol. 36 Suppl 111, 2, 2018, 78-84.
Carnevale C, Arancibia-Tagle D, Sarría-Echegaray P, Til-Pérez G, Tomás-Barberán M. Head and Neck Manifestations of Granulomatosis with Polyangiitis: A Retrospective analysis of 19 Patients and Review of the Literature. Int Arch Otorhinolaryngol. 23, 2, 2019, 165-71.
De Souza FH, Radu Halpern AS, Valente Barbas CS, Shinjo SK. Wegener's granulomatosis: experience from a Brazilian tertiary center. Clin Rheumatol. 29, 8, 2010, 855-60.
Felicetti M, Cazzador D, Padoan R, et al. Ear, nose and throat involvement in granulomatosis with polyangiitis: how it presents and how it determines disease severity and long-term outcomes. Clin Rheumatol. 37, 4, 2018, 1075-1083.
Ben Ghorbel I, Belfeki N, Baouendi N, Ben Salem T, Houman MH. Granulomatosis with polyangiitis in Tunisia. Reumatismo. 69, 1, 2017, 23-29.
Martinez Del Pero M, Rasmussen N, Chaudhry A, Jani P, Jayne D. Structured clinical assessment of the ear, nose and throat in patients with granulomatosis with polyangiitis (Wegener’s). Eur Arch Otorhinolaryngol. 270, 1, 2013, 345-354.
Rasmussen N. Management of the ear, nose, and throat manifestations of Wegenergranulomatosis: an otorhinolaryngologist's perspective. Curr Opin Rheumatol. 13, 1, 2001, 3-11.
Almouhawis HA, Leao JC, Fedele S, Porter SR. Wegener's granulomatosis: areview of clinical features and an update in diagnosis and treatment. J Oral Pathol Med. 42, 7, 2013, 507-16.
Safavi Naini A, Ghorbani J, Montazer Lotfe Elahi S, Beigomi M. Otologic manifestations and progression in patients with Wegener's granulomatosis: A survey in 55 Patients. Iran J Otorhinolaryngol. 29, 95, 2017, 327-331.
Greco A, Marinelli C, Fusconi M, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 29, 2, 2016, 151-9.
Wojciechowska J, Krajewski W, Krajewski P, Kręcicki T. Granulomatosis With Polyangiitis in Otolaryngologist Practice: A Review of Current Knowledge. Clin Exp Otorhinolaryngol. 9, 1, 2016, 8-13.
El-Mateen Moussa A and Abou-Elhmd KA. Wegener’s granulomatosis presenting as mastoiditis. Annals of Otology Rhinology and Laryngology. 107, 7, 1998, 560-563.
M. Trimarchi, R. A. Sinico, R. Teggi, M. Bussi, U. Specks, P. L. Meroni. Otorhinolaryngological manifestations in granulomatosis with polyangiitis (Wegener's). Autoimmunity Reviews. 12, 4, 2013, 501-5.
D'Anza B, Langford CA, Sindwani R. Sinonasal imaging findings in granulomatosis with polyangiitis (Wegener granulomatosis): A systematic review. Am J Rhinol Allergy. 31, 1, 2017, 16-21.
Lohrmann C, Uhl M, Warnatz K, Kotter E, Ghanem N, Langer M. Sinonasal computed tomography in patients with Wegener's granulomatosis. J Comput Assist Tomogr. 30, 1, 2006, 122-5.
Lally L, Lebovics RS, Huang WT, Spiera RF. Effectiveness of rituximab for the Otolaryngologic manifestations of granulomatosis with polyangiitis (Wegener's). Arthritis Care Res (Hoboken). 66, 9, 2014, 1403-9.
Rahmattulla C, de Lind van Wijngarden RA, Berden AE, Hauer HA, Flobmann O, Jayne DR, et al. Renal function and ear, nose, throat involvement in antineutrophil cytoplasmic antibody associated vasculitis: prospective data from the European Vasculitis Society clinical trials. Rheumatology (Oxford). 54, 5, 2015, 899-907.