E025 From risk to reward: a case of systemic antineutrophilic cytoplasmic antibody associated vasculitis
Authors: AlZeera Abdulla J , Gurdeep Dulay 
Published: April 2024

Background/Aims

The background and aims of this case involve a 70-year-old female patient with a history of complex medical conditions, including bronchiectasis and dementia, who initially presented with respiratory symptoms and fever. The primary aim was to understand the underlying cause of her deteriorating condition, which did not respond to initial antibiotic treatment for suspected pneumonia. Subsequent diagnostic efforts, including extensive vasculitis workup, were undertaken to identify the source of her symptoms, which led to the discovery of positive PR3 results and elevated inflammatory markers. The key goal was to determine the most effective treatment strategy, considering the patient’s unique medical history, balancing the need for immunosuppressive therapy against the potential risks of exacerbating underlying infections. The case ultimately aimed to demonstrate the successful management of the patient’s complex clinical presentation through a multidisciplinary approach, including immunosuppressive therapy and supportive care, resulting in significant improvements in her neurological and functional status.

Methods

We conducted a comprehensive assessment of a 70-year-old patient with ANCA-associated vasculitis (AAV) involving the central nervous system (CNS) and cutaneous manifestations. Lab tests targeted PR3-positive ANCA, and imaging assessed disease extent. A multidisciplinary team designed a tailored treatment plan with cyclophosphamide, closely monitoring progress. Ethical considerations were adhered to, and a literature review provided context. Data analysis evaluated treatment efficacy, leading to a discussion of lessons learned in managing complex AAV cases.

Results

We documented the remarkable trajectory of a 70-year-old patient with ANCA-associated vasculitis (AAV) presenting with central nervous system (CNS) involvement and cutaneous manifestations. With a meticulous and tailored treatment plan involving the judicious use of cyclophosphamide, we witnessed a profound recovery marked by significant improvements in both motor and cognitive function. This case underscores the importance of balancing potent immunosuppression with the imperative to curb disease progression and mitigate treatment-associated repercussions in complex AAV presentations. The patient’s restoration to baseline functioning serves as a compelling testament to the potential of precision medicine in managing intricate clinical scenarios of AAV. Our findings emphasize the need for continued awareness and consideration of individualized treatment approaches in similar cases, offering hope and insights for improved outcomes in challenging AAV presentations.

Conclusion

This case offers valuable lessons on the complexities and challenges involved in diagnosing and managing a multi-faceted condition like AAV. It emphasizes the urgent need for a timely and accurate diagnosis, especially when presented with atypical cases involving the central nervous system and cutaneous manifestations. Moreover, the case enriches our understanding of AAV and contributes insights into its nuanced management. It calls for a sophisticated, patient-centered approach, informed by cutting-edge research, to optimize patient outcomes in this challenging clinical scenario.

Disclosure

A.J. AlZeera: None. D. Gurdeep: None.