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IgA nephropathy with serum ANCA positivity: case series and literature review

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Abstract

The co-occurrence of IgA nephropathy (IgAN) and positive anti-neutrophil cytoplasmic autoantibodies (ANCA) serology is uncommon. In the present case series and literature review, we aimed to clarify the impact of ANCA on pathogenesis, clinical and histopathology presentation, and outcome in IgAN patients. We report four patients with an overlap lesion of IgAN–ANCA positive. Also, we performed a narrative review of all biopsy-proven published case series. Only 1.2% patients had ANCA in our 330-biopsy-proven IgAN cohort. We compared our data with previous reports—6 case series and 3 small retrospective studies—a total of 103 patients. All patients but one had eGFR below 15 mL/min at diagnosis. Besides rapidly decreasing eGFR, all presented with proteinuria around 1.5 g/day and dysmorphic microhematuria, suggesting glomerular inflammation. Systemic symptoms suggestive for ANCA vasculitis were seen in half of our patients, but only one patient had hemorrhagic alveolitis. Patients from our cohort responded to the intensive immunosuppressive regimens used in ANCA-positive vasculitis with renal involvement. However, in the follow-up, one patient had a relapse followed by septic shock related to immunosuppression and one patient started hemodialysis. In the review, we found that IgAN–ANCA -positive patients are characterized by vasculitis-like lesions and clinically by a rapidly progressive decline in kidney function, which was reversed by an aggressive induction immunosuppressive protocol used in ANCA vasculitis. Checking ANCA serology seems useful in patients with rapidly progressive IgAN for therapeutic and prognostic reasons.

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Data availability

Data extracted from the included studies in this review are available on request from the corresponding author.

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Funding

This study received no specific fund from any public, commercial, or not-for-profit agency.

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Author notes

  1. Gabriel Ștefan and George Terinte-Balcan have contributed equally to this work.

Authors and Affiliations

  1. “Dr. Carol Davila” Teaching Hospital of Nephrology, Romanian Renal Registry, Street Calea Grivitei, No. 4, 010731, Bucharest, Romania

    Gabriel Ștefan, Simona Stancu, Adrian Zugravu & Gabriel Mircescu

  2. Department of Nephrology, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania

    Gabriel Ștefan, Simona Stancu, Adrian Zugravu & Gabriel Mircescu

  3. Department of Cell Biology and Histology, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania

    Mihaela Gherghiceanu

  4. Ultrastructural Pathology, “Victor Babes” National Institute of Pathology, Bucharest, Romania

    George Terinte-Balcan & Mihaela Gherghiceanu

Authors
  1. Gabriel Ștefan
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  2. George Terinte-Balcan
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  4. Adrian Zugravu
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  5. Mihaela Gherghiceanu
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Contributions

GS and GTB conceived and designed the protocol for this study and drafted the manuscript; SS and AZ did the search and reviewed completeness and validity of extracted data; MG and GM assisted with revision of the manuscript and methodological design of the study. All authors approved the final version of this manuscript.

Corresponding author

Correspondence to Gabriel Ștefan.

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Conflict of interest

The authors declare no conflict of interest or competing interests.

Ethical approval

The study was approved by the institutional review board of Dr Carol Davila Teaching Hospital of Nephrology, Bucharest, Romania (approval number 2020–030).

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Informed consent was not required in view of the retrospective study design and the anonymity of the patient records reviewed.

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Ștefan, G., Terinte-Balcan, G., Stancu, S. et al. IgA nephropathy with serum ANCA positivity: case series and literature review. Rheumatol Int 41, 1347–1355 (2021). https://doi.org/10.1007/s00296-021-04888-2

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  • DOI: https://doi.org/10.1007/s00296-021-04888-2

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