WBR0466

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Author [[PageAuthor::Yazan Daaboul, M.D. (Reviewed by Yazan Daaboul, M.D. and Alison Leibowitz [1])]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Immunology
Sub Category SubCategory::Neurology, SubCategory::Renal
Prompt [[Prompt::A 40-year-old woman presents to the physician's office for an episode of double vision. The patient had a similar episode 4 weeks prior to presentation that self-resolved. Physical examination and neurological exam appear to be normal, but a cranial MRI demonstrates several periventricular plaques. Which disease share the same HLA subtype association as the patient's condition?]]
Answer A AnswerA::Pencil-in-cup deformity of end joints
Answer A Explanation [[AnswerAExp::Psoriatic arthritis is frequently characterized by pencil-in-cup deformity of end joints. Psoriatric arthritis affects approximately 10-15 % of patients with psoriasis. Psoriatic arthritis is associated with the HLA-B27 subtype. Other seronegative arthropaties, namely ankylosing spondylitis, arthritis of inflammatory bowel disease (IBD), and reactive arthritis (Reiter syndrome), are also associated with the HLA-B27 subtype.]]
Answer B AnswerB::Skip lesions and granulomatous inflammation of a bowel section
Answer B Explanation [[AnswerBExp::Crohn's disease is frequently characterized by skip lesions and granulomatous inflammation of a bowel section. Arthritis of crohn's disease is associated with HLA-B27 subtype.]]
Answer C AnswerC::Lymphocytic infiltratoin with germinal centers of the thyroid gland
Answer C Explanation [[AnswerCExp::Hashimoto thyroidis is characterized by lymphocytic infiltratoin with germinal centers of the thyroid gland. Hashimoto thyroiditis and pernicious anemia (vitamin B12 deficiency due to auto-antibodies against gastric parietal cells) are associated with HLA-DR5 subtype.]]
Answer D AnswerD::Antibodies against glomerular basement membrane
Answer D Explanation [[AnswerDExp::Goodpasture syndrome is characterized by auto-antibodies against glomerular basement membrane (anti-GBM). It is considered a pulmorenal syndrome because it affects the lungs and the kidneys' basement membranes. Goodpasture syndrome, similar to MS, is associated with HLA-DR2 subtype. Other diseases associated with HLA-DR2 subtype are hay fever and systemic lupus erythematosus (SLE).]]
Answer E AnswerE::Antibodies against gastric parietal cells
Answer E Explanation [[AnswerEExp::Pernicious anemia is characterized by auto-antibodies against gastric parietal cells. Pernicious anemia is a cause of vitamin B12 because parietal cells normally produce intrinsic factor (IF), a glycoprotein that binds to vitamin B12 and allows its migration along the GI tract before it is absorbed in the terminal ileum. Pernicious anemia and Hashimoto thyroiditis are associated with HLA-DR5.]]
Right Answer RightAnswer::D
Explanation [[Explanation::Multiple sclerosis (MS) is an autoimmune neurological disease that is characterized by episodic demyelination of oligodendrocytes in the central nervous system (CNS) and axonal destruction in the peripheral nervous system (PNS), which gradually debilitates the patient until death. MS frequently affects middle-aged Caucasian females and manifests as numerous periventricular plaques upon cranial MRI. MS is associated with HLA-DR2.

Several immunologic diseases are associated with different HLA-subtypes. The HLA-DR2 subtype is associated with MS, hay fever, systemic lupus erytheromatosus (SLE), and Goodpasture syndrome, characterized by antibodies against glomerular basement membranes (anti-GBM).
Educational Objective: The HLA-DR2 subtype is associated with MS, hay fever, systemic lupus erytheromatosus (SLE), and Goodpasture syndrome.
References: First Aid 2014 page 199]]

Approved Approved::Yes
Keyword WBRKeyword::HLA subtype, WBRKeyword::HLA-DR2, WBRKeyword::Multiple sclerosis, WBRKeyword::MS, WBRKeyword::Periventricular plaques, WBRKeyword::HLA
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