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January 28, 2023

A 28-year-old male presents to the emergency room complaining of shortness of breath and hemoptysis. He reports several months of dry cough and fatigue. On examination, blood pressure is 150/98 mmHg, and respiratory rate is 28/min. Labs are remarkable for a BUN of 30 mg/dL and creatinine of 2.9 mg/dL. Kidney biopsy confirms the diagnosis. Which of the following human leukocyte antigens (HLA) is most strongly associated with this patient’s disease?

Correct Answer: 

A. HLA-DR2

Explanation:

This patient is suffering from Goodpasture’s syndrome, characterized by pulmonary hemorrhage and glomerulonephritis. This disease is associated with major histocompatibility complex (MHC) class II antigen HLA-DR2. The DR2 antigen is also associated with allergy, multiple sclerosis, and narcolepsy. The underlying pathology in this disorder is an autoimmune attack against basement membrane antigens—it is also known as anti-glomerular basement membrane disease—explaining the often-simultaneous involvement of the lungs and kidneys. Kidney biopsy with immunohistochemical staining provides the diagnosis.

HLA-DR3 (choice B) is associated with celiac disease, insulin-dependent diabetes, and systemic lupus erythematosus.

HLA-DR4 (choice C) is associated with pemphigus vulgaris, rheumatoid arthritis (relative risk 4), and insulin-dependent diabetes mellitus.

HLA-DR5 (choice D) is associated with juvenile rheumatoid arthritis and pernicious anemia.

HLA-DR7 (choice E) is associated with psoriasis.

Quick Concepts:

Goodpasture’s syndrome

  • HLA-DR2

Summary:

Goodpasture’s syndrome is characterized by pulmonary hemorrhage and glomerulonephritis. This disease is associated with major histocompatibility complex (MHC) class II antigen HLA-DR2.

Explanation:



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