# Anti-melanoma Differentiation-Associated Gene 5 (Anti-MDA5) Dermatomyositis-Associated Interstitial Lung Disease Complicated by Pneumomediastinum: A Case Report and Literature Review

**Authors:** Shivang Chaudhary, Amulya Balagani, Muhammad Zaheer

PMC · DOI: 10.7759/cureus.64191 · 2024-07-09

## TL;DR

A rare case of a 58-year-old woman with a specific type of muscle disease complicated by severe lung disease and pneumomediastinum is reported, highlighting the importance of early diagnosis and treatment.

## Contribution

This case report adds to the limited literature on pneumomediastinum as a complication of anti-MDA5 dermatomyositis-associated interstitial lung disease.

## Key findings

- The patient showed clinical improvement after treatment with high-dose steroids and intravenous cyclophosphamide.
- Pneumomediastinum in anti-MDA5 DM is rare but associated with high mortality, requiring prompt diagnosis and aggressive therapy.

## Abstract

Anti-melanoma differentiation-associated gene 5 (anti-MDA5) dermatomyositis (DM) is a subset of amyopathic myositis and is associated with unique cutaneous manifestations and rapidly progressive interstitial lung disease (RP-ILD). A rare complication associated with high mortality is the occurrence of pneumomediastinum. We present a case of a 58-year-old female with anti-MDA5 DM-associated interstitial lung disease (ILD) complicated by pneumomediastinum. Treatment with pulse dose steroids and intravenous cyclophosphamide led to clinical improvement and resolution of the pneumomediastinum. Our case emphasizes the recognition of ILD-associated pneumomediastinum in patients with anti-MDA5 DM. Swift diagnosis and aggressive treatment are crucial due to the associated high mortality.

## Linked entities

- **Genes:** IFIH1 (interferon induced with helicase C domain 1) [NCBI Gene 64135]
- **Diseases:** dermatomyositis (MONDO:0016367), interstitial lung disease (MONDO:0015925)

## Full-text entities

- **Diseases:** DM (MESH:D003882), Pneumomediastinum (MESH:D008478), amyopathic myositis (MESH:C538250), Dermatomyositis-Associated Interstitial Lung Disease (MESH:D017563)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Figures

5 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11315426/full.md

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Source: https://tomesphere.com/paper/PMC11315426