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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{Rim}}
|QuestionAuthor={{Rim}} {{Alison}}
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Immunology
|MainCategory=Immunology
Line 20: Line 20:
|MainCategory=Immunology
|MainCategory=Immunology
|SubCategory=Neurology, Renal
|SubCategory=Neurology, Renal
|Prompt=A 40 year old Caucasian female patient presents to her physician's office complaining of one episode of self-resolved diplopia.  The patient had a similar episode 4 weeks prior to presentation that also self-resolved.  Physical examination is unremarkable with a normal neurological exam.  Brain MRI shows several periventricular plaques.  Which of the following other disease descriptions share the same HLA subtype association as the patient's condition?
|Prompt=A 40-year-old Caucasian female presents to the physician's office with complaints of an episode of double vision.  The patient had a similar episode 4 weeks prior to presentation, which also self-resolved.  Physical examination and neurological exam appear to be normal, but a cranial MRI demonstrates several periventricular plaques.  Which of the following disease descriptions share the same HLA subtype association as the patient's condition?
|Explanation=[[Multiple sclerosis]] ([[MS]]) is an [[autoimmune]] neurological disease characterized by episodic [[demyelination]] of [[oligodendrocytes]] in the [[central nervous system]] ([[CNS]]) and axonal destruction in the [[peripheral nervous system]] ([[PNS]]) that gradually debilitate the patient until death.  MS typically affects middle-aged Caucasian females and is characterized on brain MRI by numerous periventricular plaques. MS is associated with [[HLA-DR2]]
|Explanation=[[Multiple sclerosis]] ([[MS]]), an [[autoimmune]] neurological disease, 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]].  HLA-DR2 subtype is associated with MS, [[hay fever]], [[systemic lupus erytheromatosus]] ([[SLE]]), and [[Goodpasture's syndrome]].  The latter is characterized by antibodies against glomerular basement membranes ([[anti-GBM]]).
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's syndrome]], characterized by antibodies against glomerular basement membranes ([[anti-GBM]]).
 
|EducationalObjectives= The [[HLA-DR2]] subtype is associated with multiple MS, [[hay fever]], [[systemic lupus erytheromatosus]] ([[SLE]]), and [[Goodpasture syndrome]].
|References= First Aid 2014 page 199


Educational Objective:
HLA-DR2 subtype is associated with multiple sclerosis (MS), hay fever, systemic lupus erytheromatosus (SLE), and Goodpasture's syndrome.
|AnswerA=Pencil-in-cup deformity of end joints
|AnswerA=Pencil-in-cup deformity of end joints
|AnswerAExp=Psoriatic arthritis is characterized by pencil-in-cup deformity of end joints.  Psoriatric arthritis affects approximately 30% of patients with psoriasis, a disease associated with the HLA-B27 subtype.
|AnswerAExp=[[Psoriatic arthritis]] is frequently characterized by pencil-in-cup deformity of end joints.  [[Psoriatric arthritis]] affects approximately 30% of patients with [[psoriasis]], a disease associated with the HLA-B27 subtype.
|AnswerB=Skip lesions and granulomatous inflammation of a bowel section
|AnswerB=Skip lesions and granulomatous inflammation of a bowel section
|AnswerBExp=Crohn's disease is characterized by Skip lesions and granulomatous inflammation of a bowel section.  Crohn's disease is a type of inflammatory bowel disease that is associated with HLA-B27 subtype.
|AnswerBExp=[[Crohn's disease]] is frequently characterized by skip lesions and granulomatous inflammation of a bowel section.  [[Crohn's disease]] is a type of inflammatory bowel disease that is associated with HLA-B27 subtype.
|AnswerC=Lymphocytic infiltratoin with germinal centers of the thyroid gland
|AnswerC=Lymphocytic infiltratoin with germinal centers of the thyroid gland
|AnswerCExp=Hashimoto thyroidis is characterized by ymphocytic infiltratoin with germinal centers of the thyroid gland.  Hashimoto thyroiditis is associated with HLA-DR5 subtype.
|AnswerCExp=[[Hashimoto thyroidis]], frequently characterized by lymphocytic infiltratoin with germinal centers of the thyroid gland, is associated with [[HLA-DR5]] subtype.
|AnswerD=Antibodies against glomerular basement membrane (anti-GBM)
|AnswerD=Antibodies against glomerular basement membrane (anti-GBM)
|AnswerDExp=Goodpasture's disease is characterized by Antibodies against glomerular basement membrane (anti-GBM).  Goodpasture's disease is considered a pulmorenal syndrome because it affects the lungs and the kidneys's basement membranes.  Similar to MS, it is associated with HLA-DR2 subtype.
|AnswerDExp=[[Goodpasture's syndrome]], frequently characterized by antibodies against glomerular basement membrane (anti-GBM), is considered a pulmorenal syndrome because it affects the lungs and the kidneys' basement membranes.  [[Goodpasture's syndrome]], similar to MS, is associated with HLA-DR2 subtype.
|AnswerE=Antibodies against parietal cells of the stomach
|AnswerE=Antibodies against parietal cells of the stomach
|AnswerEExp=Pernicious anemia is characterized by antibodies against parietal cells of the stomach that is an autoimmune cause of vitamin B12 deficiency.  Pernicious anemia is associated with HLA-DR5, similar to Hashimoto thyroiditis.
|AnswerEExp=[[Pernicious anemia]], frequently characterized by antibodies against parietal cells of the stomach, is an autoimmune cause of vitamin B12 deficiency.  [[Pernicious anemia]] is associated with HLA-DR5, similarly to Hashimoto thyroiditis.
|RightAnswer=D
|RightAnswer=D
|WBRKeyword=goodpasture's syndrome, goodpasture, syndrome, anti-GBM, glomerular, basement, membrane, antibody, autoimmune, HLA, subtype, HLA-DR2, multiple, sclerosis, multiple sclerosis, periventricular, plaque, periventricular plaques,
|WBRKeyword=goodpasture's syndrome, anti-GBM, glomerular, antibody, autoimmune, HLA subtype, HLA-DR2, multiple sclerosis, MS, periventricular plaque, MRI, neurological,
|Approved=No
|Approved=Yes
}}
}}

Revision as of 13:46, 28 July 2014
Author [[PageAuthor::Rim Halaby, M.D. [1] (Reviewed by Alison Leibowitz)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Immunology
Sub Category SubCategory::Neurology, SubCategory::Renal
Prompt [[Prompt::A 40-year-old Caucasian female presents to the physician's office with complaints of an episode of double vision. The patient had a similar episode 4 weeks prior to presentation, which also self-resolved. Physical examination and neurological exam appear to be normal, but a cranial MRI demonstrates several periventricular plaques. Which of the following disease descriptions 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 30% of patients with psoriasis, a disease 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. Crohn's disease is a type of inflammatory bowel disease that is associated with HLA-B27 subtype.]]
Answer C AnswerC::Lymphocytic infiltratoin with germinal centers of the thyroid gland
Answer C Explanation [[AnswerCExp::Hashimoto thyroidis, frequently characterized by lymphocytic infiltratoin with germinal centers of the thyroid gland, is associated with HLA-DR5 subtype.]]
Answer D AnswerD::Antibodies against glomerular basement membrane (anti-GBM)
Answer D Explanation [[AnswerDExp::Goodpasture's syndrome, frequently characterized by antibodies against glomerular basement membrane (anti-GBM), is considered a pulmorenal syndrome because it affects the lungs and the kidneys' basement membranes. Goodpasture's syndrome, similar to MS, is associated with HLA-DR2 subtype.]]
Answer E AnswerE::Antibodies against parietal cells of the stomach
Answer E Explanation [[AnswerEExp::Pernicious anemia, frequently characterized by antibodies against parietal cells of the stomach, is an autoimmune cause of vitamin B12 deficiency. Pernicious anemia is associated with HLA-DR5, similarly to Hashimoto thyroiditis.]]
Right Answer RightAnswer::D
Explanation [[Explanation::Multiple sclerosis (MS), an autoimmune neurological disease, 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's syndrome, characterized by antibodies against glomerular basement membranes (anti-GBM).
Educational Objective: The HLA-DR2 subtype is associated with multiple MS, hay fever, systemic lupus erytheromatosus (SLE), and Goodpasture syndrome.
References: First Aid 2014 page 199]]

Approved Approved::Yes
Keyword WBRKeyword::goodpasture's syndrome, WBRKeyword::anti-GBM, WBRKeyword::glomerular, WBRKeyword::antibody, WBRKeyword::autoimmune, WBRKeyword::HLA subtype, WBRKeyword::HLA-DR2, WBRKeyword::multiple sclerosis, WBRKeyword::MS, WBRKeyword::periventricular plaque, WBRKeyword::MRI, WBRKeyword::neurological
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