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Disorders of the Pleura |

A Fulminant Case of Anti-GBM Antibody Disease Without Renal Symptoms

Mohammed Siddiqui, DO; Surakshya Regmi, MD; Adam Skrzynski, MD; Francisco Davila, MD; K p Ravikrishnan, MD
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Internal Medicine/William Beaumont Hospital, Royal Oak, MI


Chest. 2014;146(4_MeetingAbstracts):463A. doi:10.1378/chest.1977551
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Abstract

SESSION TITLE: Miscellaneous Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Anti-glomerular basement membrane (anti-GBM) antibody disease, or Goodpasture’s syndrome is the association of diffuse lung hemorrhage and rapid progressive glomerulonephritis. We describe a fatal case of a young female patient with a unique presentation of anti-GBM antibody disease, and review current literature on pathogenesis, diagnosis, and therapy.

CASE PRESENTATION: A 26-year-old female presented with two day history of worsening dyspnea, productive cough, and chills. On labs she was noted to be severely anemic with a hemoglobin of 6.9 g/dL. She denied any recent bloody stools, hemoptysis, or hematuria. Blood, urine and respiratory cultures were negative. Basic metabolic panel, urinalysis and stool guaiac were unremarkable. Chest x-ray revealed left lower lobe consolidation. She was started on treatment of community acquired pneumonia, and received multiple units of blood transfusion for anemia. On day 3 her respiratory status rapidly declined requiring intubation. She developed massive hemoptysis and worsening hypoxia. Emergent bronchoscopy did not show active bleeding. Empirically, she was started on high dose corticosteroids for suspected vasculitis/auto-immune pneumonitis until the appropriate diagnosis could be made. She continued to decline and expired one day later. A day after her death, anti-GBM antibody returned positive. Immunofluorescence staining during autopsy confirmed our suspicion with strong markers of immunglobulin G (IgG) deposition in the glomerular basement membrane.

DISCUSSION: Anti-GBM Antibody disease is a subset of pulmonary-renal syndromes. It usually presents with rapidly declining renal function with risk for dialysis. Disease in the younger age group is explosive, with massive hemoptysis, sudden fall in hemoglobin, and hematuria. There are some case reports of primary pulmonary involvement with subclinical renal disease, but this is the first reported case of such a rapid deterioration. Therefore, emergent renal biopsy is important to confirm diagnosis. Aggressive therapy with plasmapheresis, corticosteroids, and immunosuppressive agents has dramatically improved renal prognosis, although it is unsure if it would have been effective in such a fulminant presentation.

CONCLUSIONS: It is important to have a high clinical suspicion for anti-GBM antibody disease despite minimal signs of renal involvement. As a prompt diagnosis and therapy is imperative in preventing significant morbidity and mortality.

Reference #1: Pusey CD. Anti-glomerular basement membrane disease. Kidney Int. 2003 Oct;64(4):1535-50.

Reference #2: Hudson BG1, Tryggvason K, Sundaramoorthy M, Neilson EG. Alport's syndrome, Goodpasture's syndrome, and type IV collagen. N Engl J Med. 2003 Jun 19;348(25):2543-56

Reference #3: DJ Serisier, RCW Wong, J Armstrong. Alveolar haemorrhage in anti-glomerular basement membrane disease without detectable antibodies by conventional assays. Thorax. 2006;61:636-639.

DISCLOSURE: The following authors have nothing to disclose: Mohammed Siddiqui, Surakshya Regmi, Adam Skrzynski, Francisco Davila, K p Ravikrishnan

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