Invasive Fungal Infections in ANCA-Associated Vasculitis

A new case-based review published in Clinical Rheumatology highlights the precarious situation faced by patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) when complicated by invasive fungal infections (IFIs). The review, authored by Dr. Sahana Baliga and colleagues from the PD Hinduja National Hospital and Medical Research Centre, underscores the high mortality and diagnostic difficulties associated with these concurrent conditions.

AAV, a rare inflammatory disease affecting small to medium-sized blood vessels, includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). The primary treatment for AAV involves prolonged immunosuppressive therapy to manage disease activity and prevent relapses. However, this therapy also increases susceptibility to opportunistic infections, including IFIs.

The study presents a detailed analysis of five cases where AAV patients developed IFIs, shedding light on the clinical challenges faced by healthcare providers. These cases revealed that distinguishing between AAV flares and IFIs is particularly difficult due to overlapping symptoms and similar radiological and histopathological features. For instance, one patient was initially suspected of having an AAV flare but was later diagnosed with cryptococcal pneumonia. Candidaemia was also found to be a complication in AAV, with 2.3% of study cases having this IFI.

The review reports that the prognosis for AAV patients with IFIs is generally poor. Out of the five cases discussed, two patients succumbed to their infections despite receiving antifungal therapy. The most common fungal pathogen identified was Aspergillus, followed by Cryptococcus and a case of mixed infection with Aspergillus and Mucor. The literature review included in the study indicates that the prevalence of IFIs among AAV patients ranges from 1% to 9.6%, with pulmonary aspergillosis being the most frequently observed infection.

The authors emphasize the need for high clinical suspicion and robust microbiological diagnostics to differentiate between disease activity and infection promptly. Early and accurate diagnosis is crucial for initiating appropriate treatment and improving patient outcomes, with a 66% mortality rate based on collected case reports. The study calls for further research to develop better diagnostic tools and treatment protocols to manage this dual threat effectively.

This study also highlights the importance of individualized patient management plans, considering both the risk of infection due to immunosuppression and the need to control disease activity in AAV. This nuanced approach is vital for reducing mortality and improving the quality of life for patients with this challenging condition.

For further details, the full study can be accessed in the June 2024 issue of Clinical Rheumatology.

Patient 1: a highresolution CT (HRCT) of lung showing pulmonary nodule in the right lower lobe,
b histopathology of the lung nodule showing cryptococcal spore in the background of infammatory infltrate
Invasive Fungal Infections in ANCA-Associated Vasculitis

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