Acute exacerbation of interstitial lung disease in a patient with chronic inflammatory demyelinating polyradiculoneuropathy: A case report

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune neuropathy, and its association with interstitial lung disease (ILD) is extremely uncommon. Herein, we report the case of a 65-year-old woman with a long-standing history of CIDP who presented with simultaneous relapse of CIDP and acute exacerbation of ILD. The ILD exacerbation was resistant to high-dose corticosteroids, with worsening respiratory symptoms and progressive ground-glass opacities on imaging. Intravenous immunoglobulin (IVIG), administered 10 days after steroid initiation, resulted in significant clinical and radiological improvement in both ILD and neuropathy. This is the first reported case of acute exacerbation of CIDP-related ILD successfully treated with IVIG, which suggests a potential relationship between CIDP and ILD and suggests that IVIG may be a viable therapeutic option for steroid-resistant ILD exacerbations in patients with CIDP.