P029 Idiopathic inflammatory myopathy and axonal peripheral polyneuropathy in a poorly controlled coeliac disease patient

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.

Prognostic Factors of Severe Fever with Thrombocytopenia Syndrome in South Korea

Severe fever with thrombocytopenia syndrome (SFTS), a tick-borne infectious disease, is difficult to differentiate from other common febrile diseases. Clinically distinctive features and climate variates associated with tick growth can be useful predictors for SFTS. This retrospective study (2013–2019) demonstrated the role of climatic factors as predictors of SFTS and developed a clinical scoring system for SFTS using climate variables and clinical characteristics. The presence of the SFTS virus was confirmed using reverse transcription polymerase chain reaction (RT-PCR) tests. In the univariate analysis, the SFTS-positive group was significantly associated with higher mean ambient temperature and humidity compared with the SFTS-negative group (22.5 °C vs. 18.9 °C; 77.9% vs. 70.7%, all p < 0.001). In the multivariate analysis, poor oral intake (Odds ratio [OR] 5.87, 95% CI: 2.42–8.25), lymphadenopathy (OR 7.20, 95% CI: 6.24–11.76), mean ambient temperature ≥ 20 °C (OR 4.62, 95% CI: 1.46–10.28), absolute neutrophil count ≤ 2000 cells/μL (OR 8.95, 95% CI: 2.30–21.25), C-reactive protein level ≤ 1.2 mg/dL (OR 6.42, 95% CI: 4.02–24.21), and creatinine kinase level ≥ 200 IU/L (OR 5.94, 95% CI: 1.42–24.92) were significantly associated with the SFTS-positive group. This study presents the risk factors, including ambient temperature and clinical characteristics, that physicians should consider when suspecting SFTS.

A rare cause of proximal muscle weakness: immune necrotising myopathy

Background Immune-mediated necrotising myopathies are characterised clinically by the subacute onset of proximal limb weakness, accompanied by elevated creatinine kinase levels. They are distinguished from other myopathies by the absence of prominent infiltration of the muscle with inflammatory cells in the biopsies. Case presentation A 44-year-old man presented with upper extremity weakness and dysphagia. Laboratory tests included a creatinine kinase level of 4362 U/L (normal: 52–336 U/L). Rheumatological markers were all negative. A muscle biopsy showed multiple necrotic fibres with minimal inflammatory infiltration. One gram of methylprednisolone (IV) was given, followed by 1 mg/kg of methylprednisolone daily by the oral route. Intravenous immunoglobulin (0.4 mg/kg/day) was given for five days. Muscle weakness regressed and dysphagia disappeared with treatment. The patient remains well in the 23rd month of treatment, taking 5 mg/day prednisolone and monthly intravenous immunoglobulin. Conclusion Treatment of immune-mediated necrotising myopathy can be challenging as evidence-based therapeutic options are limited. It is generally accepted that early and extensive immunosuppression, including glucocorticoids as first-line agents, may be required.

Case Report and Cohort Analysis of Drug-Induced Liver Injury Associated with Daptomycin

ABSTRACTA patient receiving daptomycin developed asymptomatic transaminitis and hyperbilirubinemia without concurrent multiorgan dysfunction or elevation of his creatinine kinase level. After ruling out other etiologies, the liver injury was attributed to daptomycin and was subsequently resolved. A single-center retrospective cohort analysis of baseline and follow-up liver function panels (n= 614) from all admissions from 2008 to 2013 during which daptomycin was administered did not reveal any other cases of probable or definite drug-induced liver injury associated with daptomycin.