# Antineutrophil Cytoplasmic Antibodies Associated Vasculitis Presenting As Neuropathy

**Authors:** Muskaan Ahlawat, Sachin Shivnitwar, Shubhangi Kanitkar, Akshata Borle, Saipriya Ande, Abhinav Reddy

PMC · DOI: 10.7759/cureus.57046 · Cureus · 2024-03-27

## TL;DR

A 68-year-old man with AAV presented with neuropathy symptoms and was successfully treated with glucocorticoids and cyclophosphamide.

## Contribution

This case highlights AAV presenting as isolated vasculitic neuropathy without typical organ involvement.

## Key findings

- The patient showed chronic axonal neuropathy with perivascular inflammation on nerve biopsy.
- Treatment with glucocorticoids and cyclophosphamide led to significant symptom improvement.
- No respiratory or renal involvement was observed in this AAV case.

## Abstract

Antineutrophil cytoplasmic antibody-related vasculitis (AAV), is a group of diseases marked by systemic symptoms and severe small vessel inflammation. The three subtypes of AAV are eosinophilic GPA (EGPA), Microscopic Polyangiitis (MPA), and Granulomatosis with Polyangiitis (GPA). The organs that get involved in the disease process are the kidneys and the upper and lower respiratory tracts, with a spectrum of neurological manifestations. Here, we present a case report of a 68-year-old man who came with complaints of tingling and numbness over bilateral lower limbs for two months accompanied by difficulty in walking and bilateral foot drop without any respiratory complaints or involvement of sensory or autonomic system who was diagnosed with AAV (c-ANCA +) on further workup. A sural Nerve biopsy was done for confirmation which was suggestive of chronic, asymmetrical axonal neuropathy with perivascular inflammation, suggestive of vasculitic neuropathy. The patient had no other organ involvement. The patient was started on glucocorticoids and cyclophosphamide therapy for 6 cycles after which his symptoms and quality of life improved drastically.

## Linked entities

- **Chemicals:** cyclophosphamide (PubChem CID 2907)
- **Diseases:** Granulomatosis with Polyangiitis (MONDO:0012105), Microscopic Polyangiitis (MONDO:0019124), vasculitic neuropathy (MONDO:0100639)

## Full-text entities

- **Genes:** PRTN3 (proteinase 3) [NCBI Gene 5657] {aka ACPA, AGP7, C-ANCA, CANCA, MBN, MBT}
- **Diseases:** axonal neuropathy (MESH:D020269), numbness (MESH:D006987), inflammation (MESH:D007249), Antineutrophil cytoplasmic antibody-related vasculitis (MESH:D056648), tingling (MESH:D010292), EGPA (MESH:D014890), respiratory (MESH:D012131), AAV (MESH:D014657), Neuropathy (MESH:D009422), MPA (MESH:D055953), foot drop (MESH:D020427)
- **Chemicals:** cyclophosphamide (MESH:D003520)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

7 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11050731/full.md

## References

8 references — full list in the complete paper: https://tomesphere.com/paper/PMC11050731/full.md

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Source: https://tomesphere.com/paper/PMC11050731