Anti-glomerular basement membrane disease associated with thin basement membrane nephropathy: A case report.

Abstract

RATIONALE:

Simultaneous occurrence of anti-glomerular basement membrane (anti-GBM) disease and thin basement membrane nephropathy (TBMN), both of which invade the type IV collagen subunits, is very rare. Here, we present the case of a 20-year-old male patient diagnosed with both anti-GBM disease and TBMN upon presenting dyspnea and hemoptysis.

PATIENT CONCERNS:

No laboratory abnormalities, except arterial hypoxemia (PaO275.4 mmHg) and microscopic hematuria, were present. Chest computed tomography revealed bilateral infiltrations in the lower lung fields; thus, administration of empirical antibiotics was initiated. Gross hemoptysis persisted nonetheless, and bronchoscopy revealed diffuse pulmonary hemorrhage with no endobronchial lesions. Broncho-alveolar lavage excluded bacterial pneumonia, tuberculosis, and fungal infection.

DIAGNOSIS:

Enzyme-linked immunosorbent assay of his serum was positive for anti-GBM antibody (95.1 U/mL). Human leukocyte antigen (HLA) test was positive for both HLA-DR15/-DR04. Other than diffuse thinning of the GBM (average thickness, 220 nm), index kidney biopsy did not demonstrate any specific abnormalities such as crescent formation.

INTERVENTIONS:

Methylprednisolone was administered intravenously for 7 consecutive days (500 mg/day), followed by the daily dose of oral prednisolone (80 mg). Cyclophosphamide was also orally administered every day for 3 months (250 mg/day). Following 6 sessions of plasmapheresis, the anti-GBM antibody in serum became negative.

OUTCOMES:

There was no clinical evidence suggesting recurrence of pulmonary hemorrhage or azotemia during hospitalization and 12-month follow-up period. Twelve months after hospital discharge, oral prednisolone was discontinued.

LESSONS:

The patients with concurrent anti-GBM disease and TBMN will have a favorable prognosis after proper therapy. However, further research is needed to elucidate the pathogenesis and long-term outcome of the comorbidity of these 2 diseases.

• Authors: Chi Young Jung, Sun-Jae Lee, Min-Kyung Kim, Dong Jik Ahn, In Hee Lee
• Journal: Medicine (Medicine (Baltimore)) Vol. 100 Issue 20 Pg. e26095 (May 21 2021) ISSN: 1536-5964 [Electronic] United States
• PMID: 34011133 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

Topics

• Anti-Glomerular Basement Membrane Disease (complications, diagnosis, therapy)
• Glomerular Basement Membrane (diagnostic imaging, pathology)
• Humans
• Kidney Diseases (complications, diagnosis, therapy)
• Male
• Young Adult