Manifestation Of Granulomatosis Research Articles

Gastro-intestinal Manifestations in Granulomatosis with Polyangiitis: A Less Common Encountered Entity with Brief Review.

Gastro-intestinal Manifestations in Granulomatosis with Polyangiitis: A Less Common Encountered Entity with Brief Review.

Prolonged conjunctivitis mimicking nodular episcleritis as a manifestation of granulomatosis with polyangiitis—a case report

Prolonged conjunctivitis mimicking nodular episcleritis as a manifestation of granulomatosis with polyangiitis—a case report

Right atrial myxoma as the first manifestation of granulomatosis with polyangiitis, and a possible association with vascular endothelial growth factor (VEGF) and interleukin 6 (IL-6): a case report and review of the literature

BackgroundGranulomatosis with polyangiitis and myxomas are rare conditions previously described to co-exist. Cardiac masses are often presumed to be myxomas rather than lesions of granulomatosis with polyangiitis.Case presentationWe present a review of the symptoms for the two diagnoses along with the first verified case.ConclusionsTwo possible risk factors for developing myxomas (VEGF and IL-6) are explored and discussed.

Bilateral Staged Ureteral Stenosis Secondary to Wegener’s Disease: A Case report

Wegener's disease is a rare vasculitis characterized by inflammation of the endometrium of blood vessels. Ureteral stenosis is a rare manifestation of granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis). We report the case of a 38-year-old woman with acute renal failure in which bilateral hydronephrosis due to staged ureteral stenosis was the second manifestation of the disease after necrotico-inflammatory skin lesions. Our patient had high titers of anti-proteinase 3 c-ANCA, but no upper or lower respiratory tract damage was demonstrated. Hydronephrosis and renal function improved rapidly under immunosuppressive therapy with high dose of corticosteroids. We reviewed the literature and found only ten other reported cases of Wegener's granulomatosis and intrinsic ureteral stenosis: in two cases the presenting clinical manifestation was unilateral hydronephrosis and in only three others bilateral hydronephrosis. All of these complications have developed on disease relapse except in one case where the first manifestation of vasculitis is bilateral ureteral stenosis. This case highlights the importance of including ANCA-related vasculitis in the differential diagnosis of unusual cases of unilateral or bilateral ureteral stenosis.

Diabetes insipidus as the first manifestation of granulomatosis with polyangiitis: A case report

Background: Granulomatosis with Polyangiitis (GPA) is a necrotizing granulomatosis vasculitis that may influence most organs, but posterior pituitary involvement as the first manifestation is unusual. However, there are some case reports in this regard. Case presentation: A 49-year-old menopausal woman presented with polyuria, polydipsia and weight loss over time headache, purulent nasal discharge, malaise, ear pain developed. After right myringotomy due to secretory otitis media and nasal cavity biopsy, the symptoms exacerbated. Diabetes insipidus was documented by Deprivation test. Thus, 20 microgram nasal spray DDAVP was prescribed. Pituitary MRI +/- Gad demonstrated pituitary involvement. Pituitary axis hormone evaluation was consistent with menopausal state. Chest spiral CT scan showed speculated border soft tissue mass lesion. GPA confirmed with ACR criteria and C-ANCA positive. Therefore, corticosteroid and rituximab were started. Symptoms of DI responded well to the treatment and the spray dosage was decreased. Conclusion: DI can be the first manifestation of GPA; although it is rare, it is worth to be diagnosed early and serious treatment of vasculitis should be started to prevent irreversible damage to the hypophysis cell and unnecessary procedure. Keywords: GPA; granalomatosis vasculitis; diabetes insipidus.

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

P209 Where is the air coming from? Pneumomediastinum in a patient with granulomatosis with polyangiitis

Abstract Background/Aims We present the case of a 20-year-old female, who complained of a two-day history of sudden onset of global facial swelling worse in the morning, deterioration of nasal obstruction, frontal headache, found to have emphysema in the mediastinum and the neck. She was recently diagnosed with granulomatosis with polyangiitis on the basis of mild sensorineural hearing loss, extensive nasal crusting with sanguinolent discharge, myalgia, arthralgia and weight loss (10kg). Confirmed large nasal septum perforation and associated granulation via nasoendoscopy. Histology showed granulation tissue with polymorphs in the eosinophils in addition to positive immunology: C-ANCA 1:40, PR3 28. Her current treatment includes prednisolone, bone and gastric protection. She reduced her prednisolone from 50 mg to 25 mg and her facial swelling started, which led to the initial thought to be related to her prednisolone, therefore increased to 40 mg. Methods On admission, she was afebrile, tachycardic 108 per minute, saturation 99%, mild facial swelling, airways was patent, tenderness in maxillary sinuses, cardiopulmonary and abdominal examination were unremarkable. No neurology deficit. Laboratory revealed raised white cells 25 (109/L) C-reactive protein (CRP) 45 mg/L, rest unremarkable. A CT sinuses showed extensive sinonasal mucosal thickening and sinusitis, erosion of the nasal septum and free gas in the parapharyngeal, masticator and carotid spaces bilaterally, which may be due to cellulitis or fasciitis. She was started on Co-amoxiclav. Her scans were reviewed at the radiology meeting and subsequently assess for surgical emphysema and urgent investigations for collection or perforation. Results CT head, neck and thorax with contrast were performed and demonstrated air in the mediastinum and the neck extending from the level of the carina to the base of skull. There was no obvious lung abnormality or adenopathy. She remained haemodynamically stable, without respiratory distress. Coryzal symptoms persisted but other symptoms improved since her steroids were increased. Further assessments were performed, repeat nasal endoscopy showed septal perforation, significant inflammation, unable to visualise post-nasal space. Followed by maxillofacial review, ruling out dental abscess as origin of her emphysema. Respiratory review, no pneumothorax or intrinsic lung pathology. Additionally, a gastromiro was performed which ruled out perforation. Conclusion She completed a week of intravenous antibiotics then discharged on prednisolone whilst being screened for rituximab. Unfortunately, she was readmitted with shortness of breath secondary to her severe bilateral nostril obstruction, upper airway granulation. A repeat chest XR did not show evidence of pneumomediastinum or pneumothorax. She was then started on rituximab as inpatient and currently continues reducing her prednisolone with good response. Interestingly despite thorough investigations, there was no source of air leaking found, a final diagnosis of possible pneumomediastinum as rare manifestation of granulomatosis with polyangiitis was made after excluding the other causes, few cases described. Disclosure A. Escudero Siosi: None. P. Sharma: None. A. Chan: None.

Tongue Ulcer and Hemorrhagic Paronychia as Presenting Manifestations of Granulomatosis with Polyangiitis

Tongue Ulcer and Hemorrhagic Paronychia as Presenting Manifestations of Granulomatosis with Polyangiitis

Ocular Manifestations in Granulomatosis with Polyangiitis Patients from Saudi Arabia

Eye and/or orbit involvement occurs in Granulomatosis with polyangiitis (GPA) patients frequently. The aim of our study was to describe the clinical manifestations, therapy and outcome of ocular involvement in our GPA patients. A retrospective study was conducted including patients with GPA who followed up in Rheumatology clinics during 1990-2016 at King Khalid University Hospital, Riyadh. Information relating to demographics, ocular manifestations, laboratory findings, therapy and outcome of GPA patients were noted. Ocular involvement was detected in 9 (39.1%) of the 23 GPA cases identified. The mean age of ocular GPA patients was 51.8 (range 27 - 62) years, the mean age at onset of disease was 39.6 (range 11 - 57) years and the mean duration of disease was 9.0 (range 2 - 19) years. Concomitant ear, nose, throat and sino-nasal manifestations occurred with ocular symptoms in 77.8% GPA patients. The most frequent manifestations were, eye pain (66.7%), scleritis/episcleritis (55.6%), eye redness and itching (55.6% each). Antineutrophil cytoplasmic antibodies (ANCA) were positive in 88.9% patients, 55.6% had c-ANCA and 33.3% had p-ANCA. Infections were observed in 22.2% of patients, which included pneumonia in one patient and esophageal candidiasis and bacterial meningitis in another. All patients received oral prednisolone, 44.4% received intravenous cyclophosphamide, 22.2% refractory cases received rituximab doses and the disease outcome was good. Comparison of ocular GPA with non-ocular GPA patients showed that 77.8% of ocular GPA patients had concomitant sino-nasal symptoms compared to 42.9% in non-ocular GPA patients and 22.2% of the ocular GPA patients had renal involvement compared to 64.3% in non-ocular GPA patients (p = 0.049). We found that the frequency of ocular manifestations in our GPA patients was similar to reports elsewhere, and the most frequent symptom was eye pain and scleritis/episcleritis.

P-ANCA POSITIVE GRANULOMATOSIS WITH POLYANGIITIS

SESSION TITLE: Medical Student/Resident Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: It is challenging to diagnose ANCA associated vasculitis (AAV) and specify the subtype of AAV due to the overlap in symptoms and lab findings. Diagnosis can be supported by positive ANCA titer or a biopsy that confirms vasculitis present [2]. The typical ANCA pattern associated with GPA is c-ANCA. GPA is associated with P-ANCA (MPO positive) in less than 20% of the cases and approximately 10 % are ANCA negative [1] . Otorhinolaryngologic symptoms may be the initial clinical manifestation of GPA because the upper respiratory tract is involved in 70–100% of cases. Both patients of GPA and MPA can present with tracheal complications such as tracheal stenosis although it is more common in GPA. Glottic or supraglottic stenosis is a rare manifestation of AAV [1]. We present this rare case of p ANCA associated GPA presenting as stridor. CASE PRESENTATION: A 62-year-old female presented with cough, dyspnea, weight loss and tongue sores. She had significant stridor on physical exam. CT of the neck and soft tissues showed aryepiglottic edema, subglottic stenosis and pan sinus disease. Laryngoscopy in the ED and showed poor true vocal cord mobility. She then underwent tracheostomy, in addition to tongue biopsy. Direct laryngoscopy confirmed tight vocal cords, with no evidence of subglottic stenosis and normal arytenoids. Despite that the patient had increasing oxygen requirements. Her chest CT showed increased bilateral lung opacities. Bronchoscopy showed evidence of diffuse alveolar hemorrhage and granulomatous appearance of the trachea and the main stem bronchi. Work up for vasculitis was in process, while the tongue biopsy confirmed vascular inflammation and fibrinoid necrosis. later we had positive P-ANCA titer of >1:10240, MPO 25 AU/ML, negative PR3 and Anti GBM. she was started on pulse methylprednisolone and rituximab in addition to five sessions of plasmapheresis. DISCUSSION: GPA is distinguished from MPA by the absence of granulomas in the latter. Most involved organs are lungs, kidneys and ear, nose and throat. ENT involvement occurs in more than 50% of patients with AAV and can manifest as simple crusting, chronic sinusitis and rhinorrhea, to as severe as hearing loss or oral or nasal ulcers. Early diagnosis is crucial to prevent long term complications.[1] Tracheobronchial involvement is less common in GPA and would usually present as subglottic stenosis. [1], even rare is vocal cord paralysis. Our patient was diagnosed with AAV related vocal cord paralysis, which manifested as stridor. Severe active GPA can be treated with Glucocorticoids along with chemotherapeutic agents i.e cyclophosphamide or Rituximab infusion. Plasmapheresis is also helpful as it helps remove ANCA from the blood in addition to other inflammatory mediators [3]. CONCLUSIONS: Vocal cord paralysis is a rare presenting symptom for GPA, it is important to recognize it as early treatment affects the prognosis of the patient. Reference #1: 1.Greco A, Marinelli C, Fusconi M, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2016;29(2):151–159. doi:10.1177/0394632015617063 Reference #2: 2.Wojciechowska J, Krajewski W, Krajewski P, Kręcicki T. Granulomatosis With Polyangiitis in Otolaryngologist Practice: A Review of Current Knowledge. Clin Exp Otorhinolaryngol. 2016;9(1):8–13. doi:10.21053/ceo.2016.9.1.8 Reference #3: 3.Walsh M, Merkel PA, Peh CA, et al. Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial. Trials. 2013;14:73. Published 2013 Mar 14. doi:10.1186/1745-6215-14-73 DISCLOSURES: No relevant relationships by Reena Bansal, source=Web Response No relevant relationships by Lana Hasan, source=Web Response No relevant relationships by Rajani Jagana, source=Web Response No relevant relationships by Natalya Shlyakhova, source=Web Response

Severe course of quickly progressing glomerulonephritis in a patient with ANTSA-associated vasculitis: review and case report

Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, vasculitis with vascular lesions of small and medium caliber and focal necrotizing glomerulonephritis. A frequent and one of the most formidable complications is kidney damage, which in a large number of cases leads to a complete loss of organ function and a switch to renal replacement therapy. Given the rare occurrence of this disease in the clinical work of practitioners, and their low awareness of this pathology, problems often arise with the diagnosis and treatment of patients with HPA. Due to the diversity and non-specific nature of the manifestations of the disease, a delay in diagnosis may occur. The presented case illustrates the manifestations of granulomatosis with polyangiitis in the form of severe damage to the upper respiratory tract and kidneys, the diagnosis of which was difficult due to the rarity of the disease and the multiple organ pathology. This article presents a clinical case of severe progression of rapidly progressive glomerulonephritis in a patient with ANCA-associated vasculitis, a brief review of the literature is given. Despite adequate therapy, the disease progressed mainly due to deterioration of renal function, which subsequently led to a complete loss of kidney function and the transition to treatment with chronic hemodialysis.

Recurrent Skin Ulceration: A Recherché Manifestation of Granulomatosis with Polyangiitis

Recurrent Skin Ulceration: A Recherché Manifestation of Granulomatosis with Polyangiitis

GRANULOMATOSIS WITH POLYANGIITIS PRESENTING AS BILATERAL DIFFUSE ALVEOLAR HEMORRHAGE

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Granulomatosis with Polyangiitis (GPA) most commonly occurs in older adults with multi-organ involvement including skin, kidneys and respiratory tract. Some of the pulmonary complications include dyspnea, small airway obstruction, pulmonary infiltrates, cavitary lesions and rarely, pulmonary hemorrhage. We present a case diffuse alveolar hemorrhage (DAH) as the initial presentation of GPA. CASE PRESENTATION: 57-year-old African American female with history of hypertension, diabetes mellitus, ductal carcinoma in situ, ischemic stroke, tobacco abuse and obesity presented with cough, progressive dyspnea and mild hemoptysis. On pulmonary examination, she had labored breathing and expiratory wheezes. Initial laboratory examinations were all within normal limits. A chest radiograph showed diffuse bilateral infiltrates. A high-resolution computed tomography of her chest revealed diffuse bilateral alveolar infiltrates with sparing of the lung peripheries and bases with confluent ground glass nodularity. She was empirically started on intravenous methylprednisolone and oral levofloxacin. Due to worsening respiratory symptoms and increased oxygen requirements, she was intubated. Bronchoscopy revealed bilateral alveolar hemorrhage across four dilutions. Bronchoalveolar lavage was negative for bacterial growth. Video-assisted thoracoscopic surgery (VATS) wedge biopsy showed diffuse neutrophilic inflammation, without evidence of granuloma or vasculitis. Plasmapheresis was initiated. She was successfully extubated after three sessions of plasmapheresis. Rheumatological workup returned positive for PR3 antibody. She was sarted on Rituximab for presumed GPA. She was discharged to a nursing facility. On a subsequent admission 10 days later, she was diagnosed with rapidly progressive glomerulonephritis, confirmed on renal biopsy. A skin lesion biopsy showed retiform purpura with ulcerations, consistent with medium and small vessel vasculitis. She was transitioned from rituximab to induction with cyclophosphamide and plasmapheresis was continued until she was stable and discharged back to nursing facility. DISCUSSION: This is an unfortunate but interesting initial presentation of GPA with mild hemoptysis and dyspnea resulting in alveolar hemorrhage, a rare complication of GPA. In addition, her renal involvement contributes to a worse prognosis with an estimated 10-year survival rate of 40%. CONCLUSIONS: GPA is a necrotizing systemic vasculitis that involves multiple organs, most commonly affecting skin, kidneys and lungs. However, these organs can have an array of symptoms that pose a difficulty in recognizing the disease, therefore, requiring a high clinical suspicion. DAH is a rare and life-threatening complication of GPA. Some clues to further explore DAH would include hemoptysis, dyspnea, anemia and hypoxia. Early diagnosis and initiation of treatment is important to preventing morbidity and mortality. Reference #1: Greco A, Marinelli C, Fusconi M, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2015;29(2):151-9. Reference #2: West S, Arulkumaran N, Ind PW, Pusey CD: Diffuse alveolar haemorrhage in ANCAassociated vasculitis. Intern Med 52: 5–13, 2013 DISCLOSURES: No relevant relationships by Kylee Dawson, source=Web Response No relevant relationships by Navneet Kaur, source=Web Response No relevant relationships by Sowjanya Yenigalla, source=Web Response

GRANULOMATOSIS WITH POLYANGIITIS ASSOCIATED MYOCARDITIS: A MULTI-DISCIPLINARY TEAM APPROACH

Cardiac manifestations of granulomatosis with polyangiitis (GPA) are rare. We present a case of GPA with myocarditis and arrhythmia with a multi-disciplinary approach to management. A 48 year-old male presented with dyspnea and hemoptysis. He had elevated inflammatory markers, transaminitis, kidney

Clinical significance of ocular manifestations in granulomatosis with polyangiitis: association with sinonasal involvement and damage.

Ocular involvement is present in 50-60% of granulomatosis with polyangiitis (GPA) patients and can affect any part of the ocular globe. The present study describes ophthalmologic manifestations, association with systemic symptoms, disease activity and damage in GPA. A cross-sectional study was conducted including patients with GPA who underwent rheumatologic and ophthalmologic evaluation. Demographics, comorbidities, ophthalmologic symptoms, serologic markers, radiographic studies, disease activity and damage were assessed. Descriptive statistics, correlation, univariable logistic regression analyses, Student's t, Mann-Whitney U, Chi-square and Fisher's exact tests were performed. Fifty patients were included, 60% female, the median age was 56years, disease duration 72.5months. Nineteen (38%) patients had ocular manifestations at GPA diagnosis, scleritis being the most frequent; 27 (54%) patients presented ocular involvement during follow-up, repeated scleritis and dacryocystitis being the most common manifestations. Concomitant ophthalmic and sinonasal involvement was present in 12 (24%). Ocular and ENT damage occurred in 58% and 70%, respectively. Epiphora and blurred vision were the most frequent symptoms; scleromalacia and conjunctival hyperemia (27%) the most frequent clinical abnormalities. Ocular involvement at diagnosis was associated with concomitant ocular and sinonasal involvement at follow-up (OR 4.72, 95% CI 1.17-19.01, p = 0.01). Ocular involvement at follow-up was associated with age at GPA diagnosis (OR 0.94, 95% CI 0.90-0.99, p = 0.03), VDI (OR 1.29, 95% CI 1.03-1.61, p = 0.02), and ENT damage (OR 5.27, 95% CI 1.37-20.13, p = 0.01). In GPA, ocular involvement is frequent, therefore, non-ophthalmologist clinicians should be aware of this manifestation to reduce the risk of visual morbidity and organ damage.

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

Nasal Manifestations in Granulomatosis with Polyangiitis: A Case Report and Review of the Literature

Nasal Manifestations in Granulomatosis with Polyangiitis: A Case Report and Review of the Literature

Images in Clinical Otolaryngology: Nasal and Subglottic Manifestations of Granulomatosis with Polyangiitis

Images in Clinical Otolaryngology: Nasal and Subglottic Manifestations of Granulomatosis with Polyangiitis

Ear, nose and throat involvement in granulomatosis with polyangiitis: how it presents and how it determines disease severity and long-term outcomes.

Ear, nose and throat (ENT) manifestations in granulomatosis with polyangiitis (GPA) represent the most frequent symptoms at disease onset. The aim of the study was to analyse ENT involvement at diagnosis, as well as how it could influence relapse rate, mortality and disease severity. A retrospective non-controlled cohort study was performed including all consecutive diagnosed GPA from 1996 to 2016 in two rheumatology centres of Northern Italy, focusing particularly on ENT presenting signs and symptoms at baseline. Eighty-nine patients (48.3% females) with new onset GPA were evaluated. They were mostly Caucasian (97.7%), middle aged (mean 54.5years) and more frequently anti-neutrophil cytoplasmic antibodies (ANCA) positive (78.6%) with PR3 specificity (81.4%). At diagnosis, ENT involvement was reported in 71.9% patients, second only to systemic symptoms. These patients were significantly younger at disease onset (0.013), with less frequent renal involvement (0.014) irrespectively to ANCA status, but with significantly higher Vasculitis Damage Index (VDI) (0.001). The most frequent ENT manifestation was sinonasal involvement (58.4%, 73% of which with nasal inflammation/chronic sinusitis and 48% with nasal crusting), while otologic involvement (mainly otitis media/otomastoiditis) was observed in 34.8%. ENT-GPA patients presented a higher survival rate at 5years (98.1 vs 77.7%, 0.049), and ENT involvement resulted to be an independent predictor of better outcome (OR 0.37, 95% CI 0.2-0.8, 0.019). Our data confirms that ENT involvement is not only one of the key clinical features of GPA, but also could point out a milder GPA subset with lower renal involvement and lower mortality rate, irrespectively to ANCA status.

ANCA-ASSOCIATED VASCULITIS: HETEROGENEITY OF CLINICAL MANIFESTATIONS, PROGNOSIS, CURRENT OPPORTUNITIES OF PHARMACOTHERAPY

The article highlights the new views on classification and nomenclature of systemic vasculitis, the meaning of detection of antineutrophil cytoplasmic antibodies (ANCA) in the diagnosis and prognosis of the disease. The literature data and own research regarding clinical manifestations of ANCA-associated vasculitis are presented. Analyzing the first clinical manifestations in 41 patients with granulomatosis with polyangiitis, we identified four variants of the debut: with involvement of ENT organs (n ​=21), with lung lesions without involvement of the upper respiratory tract (n=8), with skin lesions, joint involvement and fever (n=7) and other variants (n=5). Fever (76%), involvement of ENT organs (51%), skin (41%), nervous system (39%) and arthritis/arthralgia (37%) were the most frequent first manifestations of granulomatosis with polyangiitis. In comparison with the presenting features there were more often lesions of the skin (66% vs. 41%), nervous system (51% vs. 39%), kidneys (41% vs. 10%), lungs (63% vs. 30%), eyes (32% vs. 10%) and myalgia (34% vs. 12%) throughout course of disease. Among the lesions of the ENT organs, sinusitis (n=19), rhinitis (n=8) and otitis (n=6) were predominate, laryngotracheitis, sublottic stenosis, saddle-shaped deformation of the nose, destruction of the walls of the paranasal sinuses and mastoiditis were diagnosed less frequently. The latest clinical recommendations for treating patients with ANCA-associated vasculitis, including the use of immunobiological therapy are presented, as well as describing of the results of our own experience in the using of immunobiological therapy in patients with granulomatosis with polyangiitis.