Diseases with major salivary glands enlargement and orbits, nose and paranasal sinuses lesions in the practice of a rheumatologist

Objective: to analyze the nosological spectrum, demographic, clinical and laboratory characteristics of diseases with a significant enlargementof major salivary (SG) / lacrimal glands, and/or accessory organs of the eye and paranasal sinuses lesions in rheumatological practice.Patients and methods. This work includes 73 patients who underwent a complex clinical and laboratory, imaging, pathomorphological and histomolecular examination, which was necessary to establish a nosological diagnosis. In all cases, the diagnosis was confirmed pathomorphologically.Results and discussion. Sjogren's syndrome (SjS) was diagnosed in 30 (41%) patients (14 of them developed lymphoproliferative disorder, LPD, as a complication), granulomatosis with polyangiitis (GPA) – in 12 (16.4%), IgG4-related disease (IgG4-RD) – in 10 (13.7%), sarcoidosis – in 6 (8.2%), non Langerhans cell histiocytosis – in 2 (2.7%), AL-amyloidosis – in 1 (1.4%), Warthin's tumor – in 1 (1.4%), chronic atrophic rhinitis – in 1 (1.4%), infectious lesions – in 3 (4.1%) (HIV-associated – in 2, dirofilariasis – in 1), idiopathic inflammatory pseudotumor – in 6 (8.2%). In 1 (1.4%) patient, the diagnosis could not be established.A massive increase of major SG was observed in 46 patients, more often (in 28 cases) with SjS with LPD or without it, with IgG4-RD (in 7) and sarcoidosis (in 6). Orbital lesions were observed in 18 patients: in 7 with IgG4-RD, in 5 with idiopathic inflammatory pseudotumor, in 2 with sarcoidosis, in 2 with GPA, and in 1 each with non Langerhans cell histiocytosis and dirofilariasis. Nasal lesions in the form of chronic rhinosinusitis with or without nasal septum perforation, were found in 18 patients, 12 of whom suffered GPA and 6 – IgG4-RD.Two algorithms, that can facilitate the choice of additional studies and the direction of diagnostic search have been proposed for practicing rheumatologists.Conclusion. Taking into account the possible similarity of clinical manifestations of the diseases with the formation of mass-like tissue, the differential diagnosis should be based on pathomorphological study.

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

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.

Nasal Septal Perforation: Experience of Management

Introduction Nasal septal perforation is the loss of composite tissue comprising the mucosa, bone or cartilage structures that form the nasal septum. Nasal septum perforation has many causes. Though it may be idiopathic, the most common causes are iatrogenic like nasal surgeries. Among other reasons are septal hematoma, nasal picking habit, nasal cauterization due to nosebleeds, nasotracheal intubation, cocaine use, vasculitis, inflammatory diseases such as sarcoidosis, This study aims to review the approach to management of patients with nasal septal perforation who underwent repair of the perforation in a tertiary clinic, in the light of current literature. Materials and Methods In this study, the records of 27 patients who were diagnosed with nasal septal perforation and treated surgically in a tertiary clinic, between January 2015 and June 2019 were reviewed retrospectively. Results The successful closure rate of perforations was 74%. In 4 of 7 patients whose perforations were not completely closed, the perforation size was larger than 2 cm in diameter. Conclusion Successful repair of nasal septal perforation depends largely on the cause, location, size of the perforation, cartilage bone tissue on the perforation edges, surgical technique and the surgeon's experience.

Cocaine-induced midline destruction

ABSTRACT In patients presenting with nasal septum perforation, the differential diagnosis between ANCA-associated vasculitis and cocaine-induced midline destruction (CIMD) can be challenging. We describe the case of a 28-year old man who presented with a nasal septum perforation. He admitted the use of cocaine and showed no other symptoms of systemic inflammation. Perinuclear anti-neutrophilic cytoplasmatic antibodies (p-ANCAs) came back positive, as did anti-proteinase 3-antibodies. Further testing revealed antibodies to human neutrophil elastase (HNE), typically found in CIMD but rarely in ANCA-associated vasculitis. The combination of an atypical ANCA-pattern and the detection of HNE-antibodies led to the diagnosis of CIMD. In conclusion, HNE antibodies can be used to distinguish between CIMD and ANCA-associated vasculitis.

Granulomatosis with polyangiitis presenting with unilateral facial nerve palsy and nasal septum perforation

Granulomatosis with polyangiitis (GPA) is a necrotising vasculitis of unknown cause that has several systemic manifestations. The disease is characterised by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can involve the central nervous system. This case report presents a case of GPA with facial nerve palsy as the first manifestation of the disease, which has been rarely reported in the medical literature.

Experience of using the P. Castelnuovo flap in the postoperative nasal septal perforation repair

Postoperative nasal septum perforation (PNSP) is a complication of nasal septum (NS) surgery with an incidence of 1–8%. The effectiveness of surgical treatment ranges from 52 to 92%. In world practice, Castelnuovo operation is actively used, while the effectiveness of this technique in the treatment of PNSP requires a comprehensive study. Objectives: To assess the effectiveness of the method of closing the PNSP with a Castelnuovo flap. Materials and methods: 26 patients with PNSP were operated on. The number of patients depending on the area (S) of the PNSP: S <1 cm2 – 2, S 1–2 cm2 – 21, S > 2 cm2 – 3. In the postoperative period, cytological examination of smears from the NS surface was carried out (3rd, 6th, 10th, 15th, 20th days), questionnaire survey NOSE and assessment of mucociliary transport of the mucous membrane (MM) of the nasal cavity (10th, 15th, 20th, 25th and 30th, 40th 60th day), the presence of relapses of PNSP within 1 year were performed. Results: pronounced leukocyte infiltration and bacterial contamination of the wound were revealed up to 10 days, reparative processes were not expressed until 15 days. The indicators of mucociliary transport were brought back to normal by the 30th day (21,25 ± 1,90 minutes), the results of the NOSE questionnaire by the 60th day were 22.12 points, which corresponded to the zone of comfortable breathing. In all 26 patients, intraoperatively complete closure of the PNSP was performed. There were 7 recurrences of PNSP: 3 in the interval of 16–30 days, 2 – from 31 to 60 days, 1 – from 61 to 90 days, 1 – from 4 to 6 months. Synechiae between NS and inferior turbinate was found in 11,5%. The efficiency of Castelnuovo flap closure technique of PNSP was 73%. Conclusions: The results indicate a pronounced inflammation in the flap and NS tissues in the postoperative period and a change in the physiological course of the wound process in favor of healing by secondary intention.