Immunoglobulin A nephropathy associated with cyclic neutropenia

Matsukura, Hiro; Watanabe, Sumi; Ito, Yoshiaki; Kanegane, H; Miyawaki, T; Shinozaki, Koji

doi:10.5414/cnp63502

Cited by 1 publication

(6 citation statements)

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“…However, according to Trimarchi et al more studies are required in the paediatric population to determine the role of this score in terms of individualized therapy selection [ 22 ]. Obviously, Nash et al (1978) and Matsukura et al (2005) did not include data in terms of IgAN prognosis [ 15 , 16 ] in their studies, thus the Oxford classification of IgAN (MEST- score) was published in 2009, long time after both studies were published, by the International IgA Nephropathy Network and the Renal Pathology Society [ 22 ].…”

Section: Discussionmentioning

confidence: 99%

“…The formation of IgA-1-containing immune complexes results in mesangial deposition and complement activation inducing mesangial and endocapillary hypercellularity, segmental glomerulosclerosis and atrophying interstitial fibrosis [ 1 , 2 ]. Interestingly, Matsukura et al highlighted that chronic administration of G-CSF could have possibly induced the pathogenesis of IgAN causing glomerular injury [ 16 ], based on Cottle et al study results, where risks of G-CSF treatment in patients with severe chronic neutropenia were highlighted [ 23 ]. However, after reviewing the current international literature about possible correlations between G-CSF administration and glomerular diseases, it became apparent that mainly case reports are described, without any clarification of the causal link between these two conditions [ 16 , 24 – 26 ].…”

Section: Discussionmentioning

confidence: 99%

“…In both studies immunofluorescence showed strong granular staining for IgA to confirm the diagnosis of IgAN. However, CN was not genetically confirmed in either study [ 15 , 16 ]. Moreover, prognosis was not discussed in abovementioned studies.…”

Section: Introductionmentioning

confidence: 94%

“…To our knowledge, the co-occurrence of IgAN and CN in paediatric patients has been reported only twice until now [ 15 , 16 ]. Specifically, the first case of a 5-year-old girl and the second case of a 10-year-old girl with CN and concomitant IgAN were published in 1978 and 2005 by Nash et al and Matsukura et al respectively.…”

Section: Introductionmentioning

confidence: 99%

“…Moreover, prognosis was not discussed in abovementioned studies. Treatment strategy of IgAN was only described in Matsukura et al study [ 16 ]. Of note, our study provides detailed information about the simultaneous occurrence of IgAN and genetically confirmed CN for the first time in the current literature, including data about diagnosis, prognosis, pathophysiology as well as treatment of both conditions.…”

Section: Introductionmentioning

confidence: 99%

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Cyclic neutropenia and concomitant IgA nephropathy: a case report

et al. 2023

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Background IgA nephropathy (IgAN) is universally recognized as one of the most common primary glomerular diseases in all ages. Cyclic neutropenia (CN) is a rare haematologic disorder that is associated with mutations of the ELANE gene. The co-occurrence of IgAN and CN is extremely rare. This is the first case report of a patient with IgAN and genetically confirmed CN. Case presentation We report a case of a 10-year-old boy who presented with recurrent viral upper respiratory tract infections accompanied by several episodes of febrile neutropenia, haematuria, proteinuria and acute kidney injury. Upon first admission, his physical examination was unremarkable. His kidney function was impaired, whereas his urine microscopy showed evidence of macroscopic haematuria and proteinuria. Further workup showed elevated IgA. The renal histology was consistent with mesangial and endocapillary hypercellularity with mild crescentic lesions, while immunofluorescence microscopy showed IgA-positive staining, which was characteristic of IgAN. Moreover, genetic testing confirmed the clinical diagnosis of CN, therefore Granulocyte colony-stimulating factor (G-CSF) was initiated to stabilize the neutrophil count. Regarding proteinuria control, the patient was initially treated with an Angiotensin-converting-enzyme inhibitor for approximately 28 months. However, due to progressive proteinuria (> 1 g/24 h), Corticosteroids (CS) were added for a period of 6 months according to the revised 2021 KDIGO guidelines with favorable outcome. Conclusions Patients with CN are more susceptible to recurrent viral infections, which can trigger IgAN attacks. In our case CS induced remarkable proteinuria remission. The use of G-CSF contributed to the resolution of severe neutropenic episodes, viral infections and concomitant AKI episodes, contributing to better prognosis of IgAN. Further studies are mandatory to determine whether there is a genetical predisposition for IgAN in children with CN.

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