Health Technology Assessment Volume: 26, Issue: 38, Published in September 2022

The use of plasma exchange (PLEX) as adjunct therapy in the initial management of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis remains to be discussed controversially in light of present evidence. In our Viewpoint we highlight scenarios where we think that physicians might still consider the use of PLEX, such as the most severe presentation forms including diffuse alveolar haemorrhage (DAH) with hypoxaemia and acute glomerulonephritis with significantly impaired kidney function. Recent sub-analyses of the PEXIVAS trial have focused on these patient populations and have provided outcomes of these patients. It became obvious that the mortality rates of those presenting with DAH were non-significantly lower and patients with severe GN show a trend of more pronounced estimated glomerular filtration rate (eGFR) recovery in the first weeks after PLEX. Notably, more patients reach a kidney function recovery as defined as eGFR increase ≥15 ml/min/1.73 m2 from baseline to week 52 when randomised to PLEX. These beneficial outcomes need to be balanced against the increased risk of serious infections and we recommend an individualized decision-making when considering addition of PLEX in the induction therapy.

Diagnosis of anomalous intrathoracic lesions may be challenging and require a multidisciplinary approach. We present a case of granulomatosis with polyangiitis (GPA) clinically and radiologically mimicking metastatic lung cancer with a bilateral pulmonary mass, mediastinal and cervical lymph node involvement, and pleural effusion. Surgical biopsy of the thoracic lesion revealed necrotic granulomatous inflammation, and the final diagnosis was subsequently confirmed by kidney biopsy and biochemical parameters. This case illustrates how comprehensive diagnosis secures timely and relevant treatment. Systemic vasculitis may be one of the key differential diagnoses in patients with multiorgan involvement, especially with pattern-mimicking lung cancer.

For over thirty-five years, available data suggested that therapeutic plasma exchange (TPE) was a useful treatment for patients with Rapidly Progressive Glomerulonephritis (RPGN) associated with ANCA Associated Vasculitis (AAV) and elevated creatinine levels. The publication of the PEXIVAS study has challenged this conclusion. This perspective will outline the history of this issue and present our assessment of the current status.