WBR0812: Difference between revisions

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|MainCategory=Pathophysiology
|MainCategory=Pathophysiology
|SubCategory=Renal
|SubCategory=Renal
|Prompt=A 45 year old Caucasian male patient is referred by the primary care physician to the nephrologist's office for nephrotic-range proteinuria. Physical examination is remarkable for high blood pressure and lower extremity edema. Following appropriate work-up, the patient is found to have elevated levels of anti-phospholipase A2 receptor (PLA2R) antibodies. The patient undergoes a kidney biopsy that shows subepithelial deposits and capillary wall thickening with normal cellularity. Anti-PLA2R IgG antibodies and C3 deposits are also observed on immunofluorescence. What is the most likely diagnosis of this patient's condition?
|Prompt=A 45 year old Caucasian male patient is referred by the primary care physician to the nephrologist's office. The patient is found to have elevated levels of anti-phospholipase A2 receptor (PLA2R) antibodies. The patient undergoes a kidney biopsy that shows subepithelial deposits and capillary wall thickening with normal cellularity. Anti-PLA2R IgG antibodies and C3 deposits are also observed on immunofluorescence. What is the most likely clinical presentation of this patient?
|Explanation=Membranous nephropathy (MN) or membranous glomerulonephritis (MGN) is a common glomerulonephritis that usually presents with nephrotic-range proteinuria, edema, and hypertension. MN is generally classified as primary (idiopathic) or secondary to other systemic disease, such as infections, malignancies, vasculitides etc. Primary MN has been recently associated with the presence of a newly discovered phospholipase A2 receptor (PLA2R) nephrotigenic antigen on the membrane surface of glomerular podocytes and anti-PLA2R antibodies. Kidney biopsy remains the gold standard for the diagnosis of MN, showing subepithelial deposits with capillary wall thickening and IgG deposition under immunofluorescence. On electron microscopy, MN shows a "spike and dome" appearance with subepithelial deposits. While MN is widely considered a chronic condition with a waxing and waning course, it is usually self-limited in the majority of the cases. Heavy proteinuria, a significant marker of prognosis, indicates the need for intervention, usually with corticosteroids and immunosuppressive therapy.
|Explanation=Membranous nephropathy (MN) or membranous glomerulonephritis (MGN) is a common glomerulonephritis that usually presents with nephrotic-range proteinuria, edema, and hypertension. MN is generally classified as primary (idiopathic) or secondary to other systemic disease, such as infections, malignancies, vasculitides etc. Primary MN has been recently associated with the presence of a newly discovered phospholipase A2 receptor (PLA2R) nephrotigenic antigen on the membrane surface of glomerular podocytes and anti-PLA2R antibodies. Kidney biopsy remains the gold standard for the diagnosis of MN, showing subepithelial deposits with capillary wall thickening and IgG deposition under immunofluorescence. On electron microscopy, MN shows a "spike and dome" appearance with subepithelial deposits. While MN is widely considered a chronic condition with a waxing and waning course, it is usually self-limited in the majority of the cases. Heavy proteinuria, a significant marker of prognosis, indicates the need for intervention, usually with corticosteroids and immunosuppressive therapy.


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Qin W, Laurence H, Beck LH Jr, Zeng C, et al. Anti-phospholipase A2 receptor antibody in membranous nephropathy. J Am Soc Nephrol. 22:1137-1143.
Qin W, Laurence H, Beck LH Jr, Zeng C, et al. Anti-phospholipase A2 receptor antibody in membranous nephropathy. J Am Soc Nephrol. 22:1137-1143.
|AnswerA=Membranoproliferative glomerulonephritis often shows glomerular basement membrane (GBM) splitting on pathology due to mesangial ingrowth.
|AnswerA=Cola-colored urine and high blood pressure
|AnswerAExp=These are not the classical findings of membranoproliferative glomerulonephritis.
|AnswerAExp=Hematuria and hypertension are signs of nephritic syndrome, which are common findings in patients with glomerulonephritis such as IgA nephropathy, rapidly progressive glomerulonephritis. In children, post-infectious glomerulonephritis and ALport's syndrome are possible etiologies for nephritic syndrome.
|AnswerB=Membranous nephropathy
|AnswerB=Peripheral edema and frothy urine
|AnswerBExp=Membranous nephropathy is characterized by the presence of antibodies against phospholipase A2 receptor. (PLA2R)
|AnswerBExp=Membranous nephropathy is characterized by the presence of antibodies against phospholipase A2 receptor (PLA2R). Most commonly, patients present with nephrotic syndrome, which is characterized by hypoalbuminemia, peripheral edema, dyslipidemia, and albuminuria (frothy urine).
|AnswerC=IgA nephropathy
|AnswerC=Urination that awakens the patient at night
|AnswerCExp=In IgA nephropathy, 30-50% of patients have elevated IgA1 levels.
|AnswerCExp=Patients with membranous nephropathy do not classically have nocturia. Nocturia may be the first sign of renal insufficiency.
|AnswerD=Rapidly progressive glomerulonephritis
|AnswerD=Burning sensation when urinating
|AnswerDExp=These are not the classical findings of rapidly progressive glomerulonephritis (RPGN). RPGN usually presents with hematuria and rapidly worsening kidney function over only a few weeks. On pathology, RPGN is characterized classically by crescent formation.
|AnswerDExp=Dysuria is a symptom of urinary tract infection.
|AnswerE=Lupus nephritis
|AnswerE=Malar rash, pallor, and photosensitivity
|AnswerEExp=These are not the classical findings of lupus nephritis. Lupus nephritis has a wide range of presentations and several sub-types.
|AnswerEExp=These are not the classical findings of systemic lupus erythematosus. Patients might thus have lupus nephritis. Lupus nephritis has a wide range of presentations and several sub-types.
|RightAnswer=B
|RightAnswer=B
|WBRKeyword=membranous, nephropathy, PLA2R, antibodies, antibody, phospholipase, A2, receptor, receptors, subepithelial, deposits, IgG, C3, complement, glomerulonephritis, nephrotic, syndrome, proteinuria, hypertension, edema, spike, dome, immunofluorescence, renal, kidney, biopsy, light, microscopy, electron
|WBRKeyword=membranous, nephropathy, PLA2R, antibodies, antibody, phospholipase, A2, receptor, receptors, subepithelial, deposits, IgG, C3, complement, glomerulonephritis, nephrotic, syndrome, proteinuria, hypertension, edema, spike, dome, immunofluorescence, renal, kidney, biopsy, light, microscopy, electron
|Approved=No
|Approved=No
}}
}}

Revision as of 08:18, 20 November 2013
Author [[PageAuthor::Rim Halaby, M.D. [1]]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathophysiology
Sub Category SubCategory::Renal
Prompt [[Prompt::A 45 year old Caucasian male patient is referred by the primary care physician to the nephrologist's office. The patient is found to have elevated levels of anti-phospholipase A2 receptor (PLA2R) antibodies. The patient undergoes a kidney biopsy that shows subepithelial deposits and capillary wall thickening with normal cellularity. Anti-PLA2R IgG antibodies and C3 deposits are also observed on immunofluorescence. What is the most likely clinical presentation of this patient?]]
Answer A AnswerA::Cola-colored urine and high blood pressure
Answer A Explanation [[AnswerAExp::Hematuria and hypertension are signs of nephritic syndrome, which are common findings in patients with glomerulonephritis such as IgA nephropathy, rapidly progressive glomerulonephritis. In children, post-infectious glomerulonephritis and ALport's syndrome are possible etiologies for nephritic syndrome.]]
Answer B AnswerB::Peripheral edema and frothy urine
Answer B Explanation [[AnswerBExp::Membranous nephropathy is characterized by the presence of antibodies against phospholipase A2 receptor (PLA2R). Most commonly, patients present with nephrotic syndrome, which is characterized by hypoalbuminemia, peripheral edema, dyslipidemia, and albuminuria (frothy urine).]]
Answer C AnswerC::Urination that awakens the patient at night
Answer C Explanation AnswerCExp::Patients with membranous nephropathy do not classically have nocturia. Nocturia may be the first sign of renal insufficiency.
Answer D AnswerD::Burning sensation when urinating
Answer D Explanation AnswerDExp::Dysuria is a symptom of urinary tract infection.
Answer E AnswerE::Malar rash, pallor, and photosensitivity
Answer E Explanation AnswerEExp::These are not the classical findings of systemic lupus erythematosus. Patients might thus have lupus nephritis. Lupus nephritis has a wide range of presentations and several sub-types.
Right Answer RightAnswer::B
Explanation [[Explanation::Membranous nephropathy (MN) or membranous glomerulonephritis (MGN) is a common glomerulonephritis that usually presents with nephrotic-range proteinuria, edema, and hypertension. MN is generally classified as primary (idiopathic) or secondary to other systemic disease, such as infections, malignancies, vasculitides etc. Primary MN has been recently associated with the presence of a newly discovered phospholipase A2 receptor (PLA2R) nephrotigenic antigen on the membrane surface of glomerular podocytes and anti-PLA2R antibodies. Kidney biopsy remains the gold standard for the diagnosis of MN, showing subepithelial deposits with capillary wall thickening and IgG deposition under immunofluorescence. On electron microscopy, MN shows a "spike and dome" appearance with subepithelial deposits. While MN is widely considered a chronic condition with a waxing and waning course, it is usually self-limited in the majority of the cases. Heavy proteinuria, a significant marker of prognosis, indicates the need for intervention, usually with corticosteroids and immunosuppressive therapy.

Educational Objective: Anti-phospholipase A2 receptor antibodies are recently discovered to be the nephritogenic antibodies implicated in membranous nephropathy. They are classically present in sera of patients with membranous nephropathy and as IgG deposits on podocyte surfaces.

Reference: Beck LH Jr, Bonegio RG, Lambeau G, et al. M-type phospholipase A2 receptor as target antigen in idiopathic membranous nephropathy. N Engl J Med. 2009;361(1):11-21.

Qin W, Laurence H, Beck LH Jr, Zeng C, et al. Anti-phospholipase A2 receptor antibody in membranous nephropathy. J Am Soc Nephrol. 22:1137-1143.
Educational Objective:
References: ]]

Approved Approved::No
Keyword WBRKeyword::membranous, WBRKeyword::nephropathy, WBRKeyword::PLA2R, WBRKeyword::antibodies, WBRKeyword::antibody, WBRKeyword::phospholipase, WBRKeyword::A2, WBRKeyword::receptor, WBRKeyword::receptors, WBRKeyword::subepithelial, WBRKeyword::deposits, WBRKeyword::IgG, WBRKeyword::C3, WBRKeyword::complement, WBRKeyword::glomerulonephritis, WBRKeyword::nephrotic, WBRKeyword::syndrome, WBRKeyword::proteinuria, WBRKeyword::hypertension, WBRKeyword::edema, WBRKeyword::spike, WBRKeyword::dome, WBRKeyword::immunofluorescence, WBRKeyword::renal, WBRKeyword::kidney, WBRKeyword::biopsy, WBRKeyword::light, WBRKeyword::microscopy, WBRKeyword::electron
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