SUN-424 TREATMENT OF BRONCHIECTASIS WITH MACROLIDE IN ANCA-ASSOCIATED VASCULITIS ACHIEVED REMISSION: A CASE REPORT

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is the most common cause of pulmonary vasculitis which most frequently manifests with diffuse alveolar haemorrhage (DAH). Proteinase 3-ANCA is most common in granulomatosis with polyangiitis (75%), whereas myeloperoxidase-ANCA occurs more frequently in patients with renal-limited vasculitis (70%). A 69-year-old female presented with two months history of lethargy and weight loss. Her medical history was significant for chronic airway disease and recurrent sinusitis. She takes regular inhaled corticosteroid and salbutamol as required. Pre-hospital investigations revealed areas of patchy pulmonary consolidation. She was treated with oral antibiotics for chest infection. On admission, she had significant renal impairment (creatinine 595umol/L, eGFR 6ml/min/1.73m2). Part of acute kidney injury investigations, a strongly positive PR3/c-ANCA with a titre of 1:2560 was reported. Induction therapy with methyl-prednisone and cyclophosphamide was commenced. Renal biopsy confirmed crescentic glomerulonephritis. On day 9 of admission, she developed haemoptysis with radiological features consistent with diffuse alveolar haemorrhage. She underwent plasma exchanges. The admission was also complicated by cardiopulmonary arrest, gastrointestinal haemorrhage and CMV colitis with prolonged recovery. During twelve-weeks of hospital admission she received four doses of rituximab. She then maintained on azathioprine and prednisone. She was readmitted with hyperkalaemia, worsening renal function with rising inflammatory markers. Trimethroprim-sulphamethoxasole prophylaxis was ceased with resultant improvement in renal function. Urinalysis did not suggest renal relapse. These episodes were treated temporarily increasing prednisone to restore remission. Positron emission tomography (PET) scan was organised to assess the disease activity, which showed inflammatory foci in both lungs. Infective screen remained negative. She then was started on macrolide (azithromycin) for bronchiectasis. Since then, inflammatory markers returned to normal and she remained well with stable renal function. View Large Image Figure ViewerDownload Hi-res image Download (PPT) This case demonstrated the necessity of multiple immunomodulatory therapies for severe AAV. Treatment of relapses remains challenging especially for frequent relapses which required intensification of immunosuppressive regimen.