CRA29 CASE SERIES ON ANCA-ASSOCIATED VASCULITIS: INSIGHTS INTO LUNG INVOLVEMENT AND DIAGNOSTIC COMPLEXITIES

Shoban Raj Vasudayan1, Tan CY1, Sumitra Appava1, Noorul Afidza1 , Mona Zaria1 ,Jamalul Azizi1
1 Hospital Sultan Idris Shah Serdang.

Introduction

ANCA-associated vasculitis (AAV) is an autoimmune disorder causing small blood vessel inflammation, destruction, and necrosis. 

Case 1:

A 48 years old woman experienced fever and cough for a month despite multiple courses of antibiotics for pneumonia. Bronchoscopy revealed erythema and edema in the left lingular segment, but tests for pathogens were negative. Her condition worsened despite antibiotics, and a CT scan revealed worsening consolidation. Repeated laboratory tests showed elevated inflammatory markers, and a positive anti-PR3 antibody test, indicating vasculitis. The patient responded well to steroid treatment, and a kidney biopsy confirmed granulomatosis with polyangiitis (GPA).

Case 2: 

A 70 year old man with a productive cough and fever  was initially treated for pneumonia. His condition rapidly deteriorated due to haemoptysis, requiring intubation. CT scan confirmed diffuse alveolar haemorrhage (DAH) and he developed rapidly progressive renal failure, necessitating  dialysis. Blood tests revealed elevated myeloperoxidase (MPO) titres and a diagnosis of microscopic polyangiitis (MPA) was made. Initial treatment with high-dose steroids and multiple rounds of plasma exchange resulted in favourable clinical, radiological, and ventilatory improvement. Unfortunately, the patient succumbed to another episode of life-threatening haemoptysis.

Case 3:

A 59-year-old man presented with chronic cough, initially treated for pneumonia. He developed worsening dyspnea and acute renal failure requiring dialysis. Laboratory findings revealed elevated eosinophil counts and MPO levels. Imaging studies showed bilateral lung consolidation with concurrent pulmonary edema. He met the criteria for Eosinophilic Granulomatosis with Polyangiitis (EGPA) and was started on immunosuppressive therapy with plasma exchange. Despite aggressive treatment, his condition deteriorated, ultimately resulting in death due to gastrointestinal bleeding.

Conclusion

ANCA-positive vasculitis with pulmonary involvement presents with multifaceted manifestations.