Case Presentation: A 52-year-old female with a medical history of chronic sinusitis and recurrent pneumonia secondary to granulomatous disease presents with new onset AKI, worsening fatigue, malaise, weight loss, and transaminitis. She was given Solu-medrol, transitioned to prednisone with improvement. Chest X-ray revealed a patchy airspace opacity in the right middle lobe, concerning for pneumonia after ruling out alveolar hemorrhage and pneumothorax. Patient was started on rituximab and transfused 1 unit of blood, per rheumatology recommendation. On hospital day (HD) 4, patient presented with rapid increase in creatinine (5.9, up from 3.14), oliguria, and hyperkalemia and was transferred to ICU for PLEX initiation. Patient underwent a total of 5 PLEX. Patient was started on rituximab and avacopan, due to concern for GPA. Patient presented to the ED 5 days post discharge with hemoptysis and was admitted to the ICU for concern for spontaneous alveolar hemorrhage. She received 3 units of pRBCs and CRRT was initiated due to concern for an AKI, with improvement. On HD4, patient underwent a kidney biopsy which revealed signs consistent with diffuse crescenteric pauci immune glomerulonephritis. The next day (HD5), patient improved. PLEX underwent another 3 sessions per Medicine recommendations. She had a resolution of hemoptysis with steroids and PLEX and was discharged on HD9.
Discussion: Granulomatosis with polyangiitis (GPA; formerly Wegener’s) is a subtype of rapidly progressive (crescenteric) glomerulonephritis. A rapidly deteriorating renal disease characterized by a positive c-ANCA/PR3-ANCA mainly affecting the small-sized arteries. It can most commonly affect the upper and lower respiratory tracts as well as the skin (Jennette et al, 1994). Patients usually present with nonspecific symptoms, hemoptysis, interstitial lung disease, urinary abnormalities (urine sediment, proteinuria, hematuria), purpuric lesion in skin, optic neuropathy, and uveitis (Falk et al, 1990; Fechner et al, 2002; Gomez-Puerta et al, 2009). There are multiple treatment options for GPA which include pulse methylprednisolone with daily oral prednisone, oral or IV rituximab or cyclophosphamide. Plasmapheresis (PLEX) is widely used yet controversial. Here, we present a case involving the use of PLEX on a patient with GPA presenting with granulomatous lung disease and acute kidney injury (AKI).
Conclusions: The role of PLEX has been advocated for patients with diffuse alveolar hemorrhage and GPA. GPA is characterized by an antibody that triggers activation of primed neutrophils and monocytes, resulting in vessel injury and activation of alternative complement pathway. PLEX clears these pathogenic autoantibodies rapidly. However, the benefits of PLEX in combination with glucocorticoids and cyclophosphamide or rituximab is not fully agreed among healthcare providers (Derebail, 2021). The use of PLEX is highly controversial and its statistical significance is still dubious. Though it may afford short-term benefits, some providers prefer administering PLEX following prognosis of kidney injury while others provide initial immunosuppressant therapy before using PLEX. Further its benefits are questioned in patients with GPA and pulmonary hemorrhage and/or AKI due to its unknown beneficial significance. Its further analysis and mechanism of action in GPA patients with pulmonary hemorrhage and/or severe kidney injury is imperative for a mainstay of therapy.