ANCA ASSOCIATED VASCULITIS IN A PATIENT WITH KNOWN SCLERODERMA – A RARE OCCURRENCE.

Dr Vishwas Raghunath¹, Dr  Kevin YC Su¹, Dr Faisal Ameer¹, A/Prof Sree Krishna Venuthurupalli¹

¹West Moreton Hospital & Health Service, Queensland Health, Ipswich , Australia

Background: The occurrence of ANCA-associated vasculitis (AAV) in patients with known systemic sclerosis has only been rarely reported. Both the conditions affect the kidney and can cause progressive kidney disease but have different diagnostic and management strategies.

Case Report: We present the case of a 52-year lady who was known to have scleroderma manifesting as polyarthritis, Raynaud’s phenomena, telangiectasia, oesophageal dysmotility, and interstitial lung disease. She presented to the emergency with haemoptysis (CT chest showing focal alveolar haemorrhage), oliguric acute kidney injury (AKI with creatinine 372 umol/L and an active urinary sediment) and hypertension after an upper respiratory tract infection. As the clinical picture was not consistent with scleroderma renal crisis, she had an urgent kidney biopsy with empiric methylprednisolone cover while awaiting a glomerulonephritis screen. She had elevated MPO ANCA titres (740 CU), and the biopsy revealed crescentic focally necrotising pauci-immune glomerulonephritis. She was commenced on oral cyclophosphamide and high dose prednisolone with multidisciplinary input. She improved without the need for dialysis support. She had a creatinine of 120-140umol/L at 3 months and 90-120umol/L at 6 months. Her ANCA titres became undetectable at 6mo. She received rituximab at 12 months after completing the appropriate COVID-19 vaccination course. She remains in remission with residual chronic kidney disease stage G3aA2 on low dose steroids and continues to see the teams in the outpatient clinic.

Conclusion: This case of ANCA-vasculitis in a patient with underlying complex connective tissue disease, manifesting as acute pulmonary-renal syndrome, is a rare occurrence. The finding of AAV in this case significantly altered management and prognosis. It highlights the need for collaborative management in unusual presentations to minimise morbidity and mortality.

Biography:

Dr Vishwas Raghunath is a nephrologist and obstetric physician at West Moreton Hospital and Health Service. He is part of the West Moreton Kidney Health service and the High-Risk Pregnancy Service, managing a range of kidney health issues and complex medical conditions in pregnancy.