A RARE CASE OF SERONEGATIVE PULMONARY RENAL SYNDROME

Abstract

SESSION TITLE: Critical Systemic Disease Case Report PostersSESSION TYPE: Case Report PostersPRESENTED ON: 10/19/2022 12:45 pm - 01:45 pmINTRODUCTION: Pulmonary-renal syndrome (PRS) is characterized by diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis (RPGN) most commonly due to autoimmune etiologies. In most cases, autoantibodies can be detected. PRS becomes challenging to diagnose in seronegative cases.CASE PRESENTATION: A 58-year-old male presented with hemoptysis, hematuria, and dyspnea. Lab results showed creatinine 5.4 mg/dL, hemoglobin 7 gm/dl, erythrocyte sedimentation rate of 65 mm/hr, C-reactive protein of 163 mg/L, and urinalysis with proteinuria and hematuria. Chest X-ray showed diffuse alveolar opacities. Computed tomography of the chest showed dense bilateral pulmonary consolidation and mediastinal lymphadenopathy.The patient's respiratory status, anemia, and renal function worsened, and was intubated. Bronchoscopy showed blood on all bronchial walls. Serial bronchoalveolar lavage revealed progressively bloodier fluid requiring blood transfusions. Auto-immune work-up including ANCA, anti-myeloperoxidase, anti-nuclear, anti-glomerular basement membrane, anti-phospholipid, and anti-cardiolipin antibodies were negative. C3, C4, serum protein electrophoresis, and kappa-lambda ratio were within normal limits.Due to clinical suspicion for vasculitis, renal biopsy was obtained. Pathology showed pauci-immune glomerulonephritis with crescent formation involving 70% of the sampled glomeruli, consistent with seronegative ANCA vasculitis. He was started on pulse-dose steroids, plasmapheresis, and cyclophosphamide for treatment. The patient's respiratory status improved, and he was successfully extubated.DISCUSSION: PRS is the concurrent presence of glomerulonephritis-induced kidney failure and respiratory failure due to diffuse alveolar hemorrhage. The most common etiologies include Good-Pasture syndrome, systemic lupus erythematosus, granulomatosis with polyangiitis, microscopic polyangiitis, and less commonly drug-induced vasculitis. Antibody-mediated injury accounts for >90% of cases, making seronegative PRS a rare presentation. In addition to the absence of serum auto-antibodies, diagnosis of seronegative PRS constitutes rapidly progressive glomerulonephritis without antibody deposition.PRS is a potentially fatal condition. Standard induction-remission regimens include pulse IV methylprednisolone followed by taper once the life-threatening condition subsides. Cyclophosphamide should be added to corticosteroid therapy in critically ill patients with generalized disease. Rituximab may be used instead of cyclophosphamide. Plasma exchange is also often used, particularly in Goodpasture syndrome and certain vasculitidesCONCLUSIONS: Seronegative pulmonary-renal syndrome is a rare and life-threatening clinical condition. The absence of specific markers often leads to delay in diagnosis. It should be considered in cases where you have a high clinical suspicion for vasculitis despite negative auto-immune work-up.Reference #1: A Rare Clinical Course of Seronegative Pulmonary-Renal Syndrome M. Fröhlich-Gildhoff,1 W. J. Jabs,2 C. Berhold,1 M. K. Kuhlmann,2 U. Ketterer,1 S. Kische,1 and H. InceReference #2: H. Yamaguchi, A. Shirakami, T. Haku, et al., "Pulmonary-renal syndrome with negative ANCAs and anti-GBM antibody,” Case Reports in Nephrology, vol. 2013, Article ID 434531, 6 pages, 2013.Reference #3: C.-C. Wang, J.-C. Shiang, M.-K. Tsai et al., "Prompt plasmapheresis successfully rescue pulmonary-renal syndrome caused by ANCA-negative microscopic polyangiitis,” Clinical Rheumatology, vol. 28, no. 12, pp. 1457–1460, 2009.DISCLOSURES: No relevant relationships by Ian JacksonNo relevant relationships by Joseph NahasNo relevant relationships by Anirudh PalicherlaNo relevant relationships by Amarjyot RandhawaNo relevant relationships by Rosina Schiff SESSION TITLE: Critical Systemic Disease Case Report Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Pulmonary-renal syndrome (PRS) is characterized by diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis (RPGN) most commonly due to autoimmune etiologies. In most cases, autoantibodies can be detected. PRS becomes challenging to diagnose in seronegative cases. CASE PRESENTATION: A 58-year-old male presented with hemoptysis, hematuria, and dyspnea. Lab results showed creatinine 5.4 mg/dL, hemoglobin 7 gm/dl, erythrocyte sedimentation rate of 65 mm/hr, C-reactive protein of 163 mg/L, and urinalysis with proteinuria and hematuria. Chest X-ray showed diffuse alveolar opacities. Computed tomography of the chest showed dense bilateral pulmonary consolidation and mediastinal lymphadenopathy. The patient's respiratory status, anemia, and renal function worsened, and was intubated. Bronchoscopy showed blood on all bronchial walls. Serial bronchoalveolar lavage revealed progressively bloodier fluid requiring blood transfusions. Auto-immune work-up including ANCA, anti-myeloperoxidase, anti-nuclear, anti-glomerular basement membrane, anti-phospholipid, and anti-cardiolipin antibodies were negative. C3, C4, serum protein electrophoresis, and kappa-lambda ratio were within normal limits. Due to clinical suspicion for vasculitis, renal biopsy was obtained. Pathology showed pauci-immune glomerulonephritis with crescent formation involving 70% of the sampled glomeruli, consistent with seronegative ANCA vasculitis. He was started on pulse-dose steroids, plasmapheresis, and cyclophosphamide for treatment. The patient's respiratory status improved, and he was successfully extubated. DISCUSSION: PRS is the concurrent presence of glomerulonephritis-induced kidney failure and respiratory failure due to diffuse alveolar hemorrhage. The most common etiologies include Good-Pasture syndrome, systemic lupus erythematosus, granulomatosis with polyangiitis, microscopic polyangiitis, and less commonly drug-induced vasculitis. Antibody-mediated injury accounts for >90% of cases, making seronegative PRS a rare presentation. In addition to the absence of serum auto-antibodies, diagnosis of seronegative PRS constitutes rapidly progressive glomerulonephritis without antibody deposition. PRS is a potentially fatal condition. Standard induction-remission regimens include pulse IV methylprednisolone followed by taper once the life-threatening condition subsides. Cyclophosphamide should be added to corticosteroid therapy in critically ill patients with generalized disease. Rituximab may be used instead of cyclophosphamide. Plasma exchange is also often used, particularly in Goodpasture syndrome and certain vasculitides CONCLUSIONS: Seronegative pulmonary-renal syndrome is a rare and life-threatening clinical condition. The absence of specific markers often leads to delay in diagnosis. It should be considered in cases where you have a high clinical suspicion for vasculitis despite negative auto-immune work-up. Reference #1: A Rare Clinical Course of Seronegative Pulmonary-Renal Syndrome M. Fröhlich-Gildhoff,1 W. J. Jabs,2 C. Berhold,1 M. K. Kuhlmann,2 U. Ketterer,1 S. Kische,1 and H. Ince Reference #2: H. Yamaguchi, A. Shirakami, T. Haku, et al., "Pulmonary-renal syndrome with negative ANCAs and anti-GBM antibody,” Case Reports in Nephrology, vol. 2013, Article ID 434531, 6 pages, 2013. Reference #3: C.-C. Wang, J.-C. Shiang, M.-K. Tsai et al., "Prompt plasmapheresis successfully rescue pulmonary-renal syndrome caused by ANCA-negative microscopic polyangiitis,” Clinical Rheumatology, vol. 28, no. 12, pp. 1457–1460, 2009. DISCLOSURES: No relevant relationships by Ian Jackson No relevant relationships by Joseph Nahas No relevant relationships by Anirudh Palicherla No relevant relationships by Amarjyot Randhawa No relevant relationships by Rosina Schiff