TWO CASES OF THSD7A-ASSOCIATED MEMBRANOUS NEPHROPATHY WITH BULLOUS PEMPHIGOID

 

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TWO CASES OF THSD7A-ASSOCIATED MEMBRANOUS NEPHROPATHY WITH BULLOUS PEMPHIGOID

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Kazuki
Kobayashi
Kazuki Kobayashi k.kobayashi061201@gmail.com Showa Medical University Nephrology Tokyo Japan *
Taihei Suzuki taihei-s@med.showa-u.ac.jp Showa Medical University Nephrology Tokyo Japan -
Masataka Ueda masu-u@med.showa-u.ac.jp Showa Medical University Nephrology Tokyo Japan -
Yuki Kajio yukit@med.showa-u.ac.jp Showa Medical University Nephrology Tokyo Japan -
Hirokazu Honda hondah@med.showa-u.ac.jp Showa Medical University Nephrology Tokyo Japan -
 
 
 
 
 
 
 
 
 
 

Membranous nephropathy (MN) is one of the common causes of nephrotic syndrome in adults. The same as phospholipase A2 receptor 1 (PLA2R), thrombospondin type-1 domain-containing 7A (THSD7A) is also known as an antigen for MN. THSD7A associated MN is often secondary to other underlying diseases including cancers. 

Clinical cases of two patients with THSD7A-associated MN accompanied by bullous pemphigoid (BP).

CaseⅠ-An 83-year-old man with untreated diabetes mellitus (DM) presented with a 2-month history of blisters and edema. His laboratory data showed nephrotic syndrome (albumin: 1.7 mg/dL, proteinuria: 5.3 g/day) without renal impairment. Additional tests, including serologies for ANA, ANCA, Hepatitis B surface Ag, and Hepatitis C antibodies (Abs) were negative, and imaging analysis did not find apparent causes for MN. The patient was diagnosed as MN based on the findings of kidney biopsy. Immunofluorescent (IF) staining revealed IgG and THSD7A existed along the glomerular basement membrane (GBM). Based on the dermatologic findings and elevated serum BP180 antibody (76.6 U/mL), we considered that the patient with MN was accompanied by BP. CaseⅡ-A 73-year-old man with 10-year DM treated with linagliptin developed blisters and edema. We diagnosed the patient as BP according to the skin biopsy showing linear IgG around the basement membrane zone and high titer of serum BP180 antibodies (18.2 U/mL). Following BP, he developed nephrotic syndrome (albumin: 1.6 mg/dL, proteinuria: 7.3 g/gCr). We could not detect other causes of MN, including infectious, autoimmune, or malignant etiologies. The findings of the kidney biopsy confirmed that the nephrotic syndrome was caused by MN. The additional IF staining revealed that IgG and THSD7A were positive along GBM as in case 1.

 Although several case reports showed that co-existence of MN and BP in patients with nephrotic syndrome, to our knowledge, there has been no prior report suggesting that the responsible antigen of MN accompanied by BP was THSD7A. In both of our cases, THSD7A was detected along GBM. Further reports and studies are required to elucidate the pathogenic association between THSD7A-associated MN and BP.

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