Aggressive management of pulmonary-renal syndrome: a case report

SESSION TITLE: Diffuse Lung Disease Case Report Posters 1 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 12:00 pm - 12:45 pm INTRODUCTION: Pulmonary-renal syndrome (PRS) describes a process that results in pulmonary hemorrhage and glomerulonephritis (GN). Multiple autoimmune diseases can lead to PRS, including pauci-immune crescentic GN. CASE PRESENTATION: A 22-year-old female presented with hemoptysis and fevers for the past five days. She was tachycardic and tachypneic, and her oxygen saturation was 95% on room air. Her lab workup was remarkable for leukocytosis, anemia, peripheral eosinophilia (11%), and elevated ESR/CRP. Her C4 was also decreased, and her C3 was normal. CT angiogram of the chest showed multifocal regions of pulmonary parenchymal consolidation with cavitations bilaterally. She was admitted and started on broad-spectrum intravenous antibiotics. She underwent bronchoscopy, and serial bronchoalveolar lavage (BAL) specimens were progressively more hemorrhagic. Analysis of BAL samples showed 44% eosinophils and scant growth of Klebsiella pneumoniae. She was transferred to the ICU for increasing oxygen requirements and persistent hemoptysis requiring blood transfusions. She eventually required intubation. Autoimmune workup returned positive with elevated proteinase 3 antibody (696 AU/mL) and c-ANCA (titer 1:640). She was immediately started on pulse dose steroids, rituximab, and received six sessions of plasmapheresis (PLEX). The patient also developed an oliguric kidney injury requiring temporary hemodialysis. After aggressive treatment, she was successfully extubated. She later underwent a right kidney biopsy which showed findings consistent with pauci-immune crescentic GN and was discharged on a prolonged steroid taper. DISCUSSION: PRS requires a prompt, accurate diagnosis as mortality rates can reach 25-50% [1]. Variable pathogenic mechanisms are involved; however, 70% of cases are believed to be associated with antibodies to ANCA which lead to a rapidly progressing pauci-immune crescentic necrotizing GN [2]. Small vessel vasculitis in arterioles, venules, and alveolar capillaries eventually lead to the extravasation of blood into the alveolar space and diffuse alveolar hemorrhage (DAH). In most PRS cases, the renal damage is due to focal proliferative GN. Combined with suggestive laboratory and imaging workup, bronchoscopy is usually required to confirm the diagnosis of DAH [1]. Although renal or lung biopsy is typically preferred before starting long term immunosuppression, the severity of our patient's illness precluded biopsy before aggressive treatment. The cornerstone of treatment of ANCA associated PRS is immunosuppression, which is usually achieved by initiating pulse dose steroids. For severe disease, cyclophosphamide or rituximab should also be used to induce remission. Although PLEX is commonly used, recent trials have not shown a decrease in mortality or end stage kidney disease [3]. Long term azathioprine, rituximab, methotrexate, and mycophenolate mofetil can be used along with low dose glucocorticoids for remission maintenance. CONCLUSIONS: PRS should be high on the differential diagnosis list in appropriate clinical settings. It should be treated aggressively and swiftly with immunosuppression as the mortality rate is high with delay in treatment. REFERENCE #1: Papiris, S.A., Manali, E.D., Kalomenidis, I. et al. Bench-to-bedside review: Pulmonary–renal syndromes – an update for the intensivist. Crit Care 11, 213 (2007). https://doi.org/10.1186/cc5778 REFERENCE #2: Saladi L, Shaikh D, Saad M, et al. Pulmonary renal syndrome: A case report of diffuse alveolar hemorrhage in association with ANCA negative pauci-immune glomerulonephritis. Medicine (Baltimore). 2018 Jun;97(23):e10954. doi: 10.1097/MD.0000000000010954. PMID: 29879042; PMCID: PMC5999515. REFERENCE #3: Walsh M, Merkel PA, Peh CA, et al. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis. N Engl J Med. 2020 Feb 13;382(7):622-631. doi: 10.1056/NEJMoa1803537. PMID: 32053298; PMCID: PMC7325726. DISCLOSURES: No relevant relationships by Mohamad Al-Momani No relevant relationships by Kevin Cornwell No relevant relationships by Rami Dalbah No relevant relationships by Ahmad Othman No relevant relationships by Mohammad Qureshi No relevant relationships by Krupa Solanki