An autoimmune storm: A case based literature review of successful management of lupus-polymyositis overlap syndrome complicated by multiple organ failure

Overlap syndromes of Connective tissues diseases is a rare and under-studied disorder. This syndrome can be complicated by multiple life-threatening complications like hepatitis, pancreatitis, macrophage activation syndrome and Acute Respiratory Distress syndrome. We report a 22-year-old Hispanic male with no remarkable past medical history who presented to the hospital with abdominal pain, myalgia, arthralgia, and persistent fever for two weeks and was found to have tachycardia, pancytopenia, transaminitis and an elevated lipase. The patient was initially evaluated for acute pancreatitis and hepatitis due to binge drinking and presumptive viral infection. Infectious work up was unrevealing, and the patient continued to deteriorate. The autoimmune panel came back significant for highly elevated Antinuclear Antibody (ANA) and Anti Double Stranded DNA antibody (dsDNA), low complement, high inflammatory markers, ferritin, elevated Creatine Kinase (CK), positive Anti Mi 2 antibody, low Natural Killer (NK) cell activity and elevated CD25. Patient was subsequently diagnosed with Lupus- Polymyositis overlap syndrome. Acute lupus flare was considered as the trigger for pancreatitis and hepatitis. The hospital course was complicated by Acute Respiratory Distress Syndrome (ARDS) due to Lupus pneumonitis, encephalopathy, and Macrophage Activation Syndrome (MAS). Patient was treated successfully with steroid pulse therapy, Cyclophosphamide, plasmapheresis, artificial ventilation, and extracorporeal membrane oxygenation (ECMO). We believe that this case is unique as it represented a diagnostic challenge as the Lupus-Polymyositis overlap syndrome is a rare entity, and it initially manifested in an extensively complicated pattern which is unusual. Moreover, this case was not only a diagnostic challenge but also a treatment conundrum due to multiple life-threatening complications the patient had during this hospitalization. In this review, we intend to discuss the management of this challenging and rarely reported connective tissue disease overlap syndrome.