Kimura's Disease Associated with Membranous Nephropathy with IgG4 and Phospholipase A2 Receptor-positive Staining of the Glomerular Basement Membrane

Okura, Takafumi; Miyoshi, Katsuhiko; Irita, Jun; Enomoto, Daijiro; Nagao, Tetsuhiko; Kukida, Masayoshi; Tanino, Akari; Kudo, Kayo; Higaki, Jitsuo

doi:10.2169/internalmedicine.53.2042

Cited by 11 publications

(11 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Most such cases were reported from Asian countries, including Japan and China, reflecting the endemic regions of Kimura’s disease. Okura et al reported a case of Kimura’s disease with MN characterised by glomerular IgG4 and PLA2R positivity, providing the first evidence of PLA2R-associated MN with comorbid Kimura’s disease [ 29 ]. Similarly, the pathogenic association of THSD7A-associated MN with asthma (cases 10 to 12) remains unclear, although hypereosinophilic disorders other than Kimura’s disease have been reported in association with MN [ 30 , 31 ].…”

Section: Discussionmentioning

confidence: 99%

Clinicopathological characteristics of thrombospondin type 1 domain-containing 7A-associated membranous nephropathy

et al. 2019

View full text Add to dashboard Cite

Thrombospondin type 1 domain-containing 7A (THSD7A) is a recently identified target antigen of idiopathic membranous nephropathy (iMN). The clinicopathological characteristics of THSD7A-associated MN are poorly characterised due to low prevalence among MN patients. Among 469 consecutive cases of pathologically confirmed MN diagnosed at four centres in Japan, 14 cases were confirmed positive for THSD7A by immunohistochemistry (3.0%). The prevalence of THSD7A-associated MN tended to be higher in northern Japan. Most cases demonstrated nephrotic-range proteinuria (12/14 cases, 86%). In two patients, cancer was detected at the time of renal biopsy (small-cell carcinoma of the lung and prostatic adenocarcinoma with neuroendocrine differentiation). Both tumours were negative for THSD7A. Four patients had concurrent or previous incidence of allergic diseases, including one patient with Kimura’s disease. Pathological analysis of kidney biopsy tissue revealed slight mesangial cell proliferation in three cases and spike formation in one case. Immunofluorescence studies demonstrated that IgG subclass was mainly IgG4-dominant/codominant (12/13, 92% cases), while the case with prostatic cancer had an IgG2-dominant distribution. The immunostaining profile for components of the lectin complement pathways was not significant in three cases including two patients with malignancy. One case was dual positive for THSD7A and PLA2R. Of 10 cases with known clinical follow-up data, 6 demonstrated reduced serum creatinine and 8 presented reduced proteinuria. In summary, although the major IgG phenotype was usually IgG4-dominant/codominant, clinical background was otherwise heterogeneous. Further investigation of regional differences in THSD7A-associated MN prevalence may reveal genetic and environmental risk factor and associated pathogenic mechanisms. Electronic supplementary material The online version of this article (10.1007/s00428-019-02558-0) contains supplementary material, which is available to authorized users.

show abstract

Section: Discussionmentioning

confidence: 99%

Clinicopathological characteristics of thrombospondin type 1 domain-containing 7A-associated membranous nephropathy

et al. 2019

View full text Add to dashboard Cite

show abstract

“…There is more than one previous report of the co-presence of KD and IgG4-related disease. [ 6 ] These reports suggest that KD may be more likely to occur in patients with autoimmune or auto-inflammatory diseases. We surmise that mutated genes leading to immune disorders in these patients may contribute to the pathogenesis of KD.…”

Section: Discussionmentioning

confidence: 99%

“…Many cases of autoimmune diseases complicated with KD have been reported, including systemic lupus erythematosus (SLE), Behcet's syndrome (BD), and IgG4-related diseases. [ 4 – 6 ] To date, however, KD associated with ankylosing spondylitis (AS) has not been reported. Herein, we describe the case of a patient with familial AS who was diagnosed with KD.…”

Section: Introductionmentioning

confidence: 99%

Kimura's disease and ankylosing spondylitis

Chen

Cui

et al. 2020

Medicine

View full text Add to dashboard Cite

Rationale: Ankylosing spondylitis (AS) and Kimura's disease (KD) which is quite rare are both chronic inflammatory diseases. Recently we encountered a patient who suffered from KD and AS, and some of his family members also suffer from AS. We, therefore, investigated this unique case and conducted the family-based whole exome sequencing to explore the possible genetic alterations. Patient concerns: Here, we reported a case of a 44-year-old Chinese man with multiple painless masses all over his body and a back pain for 32 years. His uncle and sister were diagnosed with AS. Diagnosis: The diagnosis of KD was based on the patient's clinical features and the biopsy of the neck masses. The diagnosis of AS was based on the patient's clinical features, HLA-B27(+) and the radiologic changes of sacroiliac joints. The genetic test showed that ARPC1B gene which was associated with recurrent infections, auto-inflammatory changes and elevated IgE levels was mutated in this patient. Interventions: Neck masses were removed by surgery. Systemic glucocorticoid, nonsteroidal anti-inflammatory agents, combined with cyclosporine were orally administered, and Etanercept was injected subcutaneously. Outcomes: The masses disappeared rapidly after surgery combined with systemic glucocorticoid, but relapsed shortly after the therapy was discontinued. Low dose glucocorticoid, cyclosporine and Etanercept could keep both KD and AS remained long-term remission. Lessons: Our experience suggests that low dose glucocorticoid, cyclosporine and Etanercept could be beneficial for the patient with KD and AS. The mutation of ARPC1B gene in this case, which is associated with immunologic disturbance, may increase the susceptibility of KD.

show abstract

“…It has been speculated that viral infections or toxins may stimulate the release of lymphokines by altering T cell immunoregulation. These immunological triggers could result in associated renal lesions [13,17]. Regarding the relationship between eosinophilia and THSD7A-associated MN, Matsumoto et al presented two cases of THSD7A-associated MN accompanied by ALHE, a benign tumor characterized by the proliferation of plump endothelial cells [8].…”

Section: Discussionmentioning

confidence: 99%

Membranous nephropathy associated with thrombospondin type-1 domain-containing 7A (THSD7A) in an adult woman with eosinophilia

et al. 2019

View full text Add to dashboard Cite

A 30-year-old woman on steroid therapy for eosinophilia presented with nephrotic syndrome during steroid tapering. She was diagnosed with membranous nephropathy (MN) stage II-III (positive for IgG1 and IgG4) by renal biopsy. There was no evidence of secondary MN. Her urinary protein level was controlled to 0.5 g/day or less, and her eosinophil count in white blood cell differential was stabilized at less than 10% without increasing the steroid dosage. The renal specimen did not show any enhanced granular expression of PLA2R along the glomerular basement membrane, and PLA2R was not detected in the patient's serum. On retrospective analysis, enhanced granular staining for thrombospondin type-1 domain-containing 7A (THSD7A) in the glomeruli was detected in the biopsy, and anti-THSD7A IgG was detected in the serum using a commercial indirect immunofluorescence test (IFT). Based on these, the case was considered as THSD7A-associated MN with comorbid eosinophilia. The causal relationship between THSD7A-related MN and eosinophilia was unclear. However, a few cases of THSD7A-associated MN with eosinophilia have been reported, and further clarification on the relationship between THSD7A-related MN and eosinophilia is warranted.

show abstract

Kimura's Disease Associated with Membranous Nephropathy with IgG4 and Phospholipase A2 Receptor-positive Staining of the Glomerular Basement Membrane

Cited by 11 publications

References 26 publications

Clinicopathological characteristics of thrombospondin type 1 domain-containing 7A-associated membranous nephropathy

Clinicopathological characteristics of thrombospondin type 1 domain-containing 7A-associated membranous nephropathy

Kimura's disease and ankylosing spondylitis

Membranous nephropathy associated with thrombospondin type-1 domain-containing 7A (THSD7A) in an adult woman with eosinophilia

Contact Info

Product

Resources

About