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.
Background/Aims Pneumocystis jirovecii pneumonia (PJP) is a rare but frequently fatal fungal infection, which can affect patients with rheumatic diseases treated with immunosuppressants or high doses of corticosteroids. The only guidelines for primary prophylaxis of this infection are for vasculitides treated with cyclophosphamide or rituximab. The aim of this abstract is to raise awareness of this preventable infection and to highlight the urgent need to create a tailored risk assessment algorithm to identify patients at increased risk of this infection. Methods We collected information on patients with autoimmune inflammatory rheumatic diseases (AIIRD) who had final working diagnosis of PJP from January 2021 to July 2022 in Oxford University Hospitals NHS Foundation Trust. Patients were identified through infection, respiratory and rheumatology teams. Electronic notes were reviewed to collate clinical data. Results We identified 11 patients who had definite or probable PJP infection of which 7 were female. Of these, 6 were being treated for large vessel vasculitis (LVV), 3 had rheumatoid arthritis (RA) and 2 patients had connective tissue disease (CTD). Median age was 78 years (range 46-93 years). 10 patients had lymphopenia with lymphocyte count <1x10^9/L (the only patient without lymphopenia had chronic lymphocytic leukaemia (CLL)). Prednisolone was prescribed in combination with disease modifying anti-rheumatic drugs (DMARDs) in 6 patients and monotherapy (high dose) in 3 patients, whilst 2 patients were on DMARDs/biologic only. None of the patients who developed PJP had been given prophylaxis prior to the infection. All patients were treated with cotrimoxazole or atovaquone (in one case) for PJP. Unfortunately, 4 died from PJP infection. All these patients were taking 20-60mg prednisolone. Summary patient characteristics are shown in the table below. Conclusion PJP appears to be more common than is currently recognised. Prescribing PJP prophylaxis is not standard practice for patients with CTD and LVV. Consideration needs to be given to PJP prophylaxis for patients on high dose corticosteroids for a prolonged period, particularly in the presence of other risk factors. Better screening mechanisms are needed to identify those patients who will benefit most from PJP prophylaxis. Disclosure A. Verdiyeva: None. A. Escudero Siosi: None. M. Andersson: None. C. Woodrow: None. S. Dubey: None.
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