Introduction:
Anti-melanoma differentiation-associated protein 5 (MDA5) antibody-positive dermatomyositis (MDA 5 DM) is a subtype of a rare autoimmune disorder characterised by various skin manifestations and interstitial lung disease (ILD), which may progress rapidly. Most patients do not present significant muscle weakness (amyopathic dermatomyositis). Recent publications suggest a correlation between anti-MDA-5 antibodies and acute pancreatitis; thus, we present two case reports of adult patients with anti-MDA-5 dermatomyositis who also developed acute pancreatitis.

Case description:
A 52-year-old woman was diagnosed with amyopathic anti-MDA-5 positive dermatomyositis with ILD of probable paraneoplastic origin due to suspected laryngeal carcinoma, which was later excluded. One month later, she was admitted to the Emergency Department, diagnosed with acute pancreatitis (abdominal pain, elevated pancreatic enzymes levels and typical radiological findings), and treated conservatively. Differential diagnosis excluded cholelithiasis, alcohol abuse, hypertriglyceridemia, and other causes. Unfortunately, she developed multiple organ failure and died after 24 days of Intensive Care Unit hospitalisation. The second patient was a 40-year-old man also diagnosed with amyopathic anti-MDA-5 positive dermatomyositis (typical skin changes, arthritis, fever). Additionally, rapidly progressive interstitial lung disease was identified. Treatment was initiated with intravenous methylprednisolone and cyclophosphamide. However, a few days after discharge, he was re-admitted with the suspicion of acute pancreatitis (fever, vomiting, upper abdominal pain, elevated pancreatic enzymes levels) and treated conservatively. Other causes of pancreatitis were excluded. Due to the severe disease course and insufficient response to glucocorticosteroid therapy, intravenous immunoglobulins and cyclosporine A were implemented with a significant clinical improvement.

Conclusions:
The MDA-5 DM was first described in 2005. Subsequently, it was reported that this DM subtype may be associated with an increased risk of developing acute pancreatitis. However, further long-term observational studies and extended, detailed follow-ups are necessary to confirm a potential causal relationship. Moreover, other acute pancreatitis causes, such as cholelithiasis, should be excluded. Eventually, it is important to include this rare but potentially life-threatening complication in MDA-5 DM patients.