ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: PO2256

Case of Pulmonary-Renal Syndrome Involving Goodpasture Disease and Granulomatosis with Polyangiitis

Session Information

Category: Trainee Case Report

  • 1602 Pathology and Lab Medicine: Clinical

Author

  • Khan, Shabtab, AU-UGA Internal Medicine, Athens, Georgia, United States
Introduction

Wegner’s granulomatosis and Goodpasture’s disease are two rare causes of pulmonary-renal syndromes syndromes. Both have similar presentation and differentiation is crucial for early management. WG is a systemic vasculitis syndrome that affects the respiratory and renal systems and associated with C-ANCA (PR3) antibodies. Goodpasture’s disease is an autoimmune condition that is characterized by rapidly progressive glomerulonephritis (RPGN) and severe alveolar hemorrhage. It is most often related to IgG antibodies against type 3 collagen in the glomerulus and renal basement membranes. It is noted that 5% of all ANCA + patients are also positive for anti-GBM and of all the anti-GBM about 1/3rd also have ANCA but that does not always correlate with active clinical disease. This leads to a significantly poor prognosis and worse renal outcomes than either disease process alone.

Case Description

We present a 59-year-old female with initial complaint of weakness, lethargy and sinus congestion. She had a history of untreated hypertension. She was noted to be hypoxic and had rales and rhonchi bilaterally. Labs revealed WBC of 35, Hg 5.6, Hct 15.5 and platelets 594. She had sodium of 98, Potassium 5.8, Bicarbonate 18, BUN of 58 and Creatinine of 8.5. Chest x-rays showed bilateral opacities resembling CAP. Further hypoxemia prompted intubation, revealing copious amounts of alveolar hemorrhage. Vasculitis was in the differential given alveolar hemorrhage and renal failure and she was promptly started on plasmapheresis, high dose IV steroids as well as cyclophosphamide. Complement levels and immunofixation were normal as were studies for lupus and hepatitis B and C. She had + ANA, Anti-GBM and c-ANCA antibodies. Renal biopsy showed predominant sclerosis and >90% focal necrotizing crescentic GN and severe interstitial scarring. Immunofluorescence (IF) demonstrated linear staining of glomerular BM as well as ANCA mediated changes.

Discussion

This case illustrates that both WG and GP can occur in the same patient. Such patients can present with CAP that rapidly deteriorates. Early recognition of pulmonary involvement was crucial to start proper treatment by plasmapheresis and immunosuppressant. Although these patients with severe glomerular involvement will be lifelong dependent on dialysis, their 1- and 2-year survival can be significantly improved with appropriate therapy and follow up.