P029 Idiopathic inflammatory myopathy and axonal peripheral polyneuropathy in a poorly controlled coeliac disease patient
Authors: Tan Yvonne , Mohamedalhadi Ammar , Wood Fiona 
Published: April 2021

Background/Aims 

The current mainstay treatment of idiopathic inflammatory myopathies (IIM) includes steroid therapy with treatment escalation to steroids -sparing immunosuppressants to maintain remission. Nevertheless, some patients may not fully recover to their normal muscle strength, while exposing to potential side effects of these drugs. We report the first case of IIM and axonal peripheral polyneuropathy in a poorly controlled coeliac patient, who made complete recovery with adherence to gluten-free diet.

Methods 

A 76-year-old white Caucasian lady, who was previously independent, presented with 6-weeks history of pain and weakness in both legs. She required the use of a wheelchair to get around as she was unable to weight bear as the result of the pain. She denied any change to regular medications, history of trauma, new rash, joint swelling, or B-symptoms. There was no history of smoking, alcohol, statins, or steroid use. She was previously diagnosed with coeliac disease 10 years ago but had opted not to adhere to a gluten-free diet due to personal preference.

Results 

On examination, there was significant muscle tenderness on palpation of bilateral lower limbs. The rest of systemic examination was unremarkable. Duodenal biopsy revealed severe villous atrophy and chronic lymphocytic and plasma cell inflammation, which reinforced the diagnosis of coeliac disease. All immunology screen including ANA, ENA, ds-DNA, myositis and vasculitis screen were negative except anti-RO-52. This patient did not have any clinical feature of Sjogren’s or systemic lupus erythematosus. EMG revealed fibrillation potentials, positive sharp waves, and myotonic discharges in the paraspinal, upper and lower limbs muscles. It also demonstrated underlying axonal peripheral polyneuropathy. Muscle biopsy showed features consistent with the diagnosis of idiopathic inflammatory myopathy. This patient was started on a gluten-free diet during admission. Interestingly, we discovered that her creatinine kinase level had improved spontaneously along with the clinical improvement of her presenting symptoms. As her symptoms have completely resolved, she was discharged home. She was never started on steroid or immunosuppressant due to her significant clinical improvement. At three-month follow up, her creatinine kinase level had normalised, and she remained in remission while adhering to a gluten-free diet.

Conclusion 

To the best of our knowledge, there is no reported case of complete clinical resolution of IIM with gluten withdrawal in coeliac disease patients, without the need of steroid or immunosuppressants. In addition, the concomitant of myopathy and polyneuropathy, as some described as neuromyositis, is extremely rare. The pathophysiology remains unclear; however, it is proposed that neuropathy could be an extra-muscular manifestation of IIM.The clinical remission of IIM in our patient with adherence to a gluten-free diet provide an alternative treatment option to the current recommended regimes of steroid and immunosuppressants. Such non-pharmacological approach is favourable, especially in the current SARS-CoV-2 pandemic.

Disclosure 

Y. Tan: None. A. Mohamedalhadi: None. F. Wood: None.