Search

Sporadic inclusion body myositis (sIBM) has been reported to occur in association with autoimmune diseases and in particular, primary Sjogren's syndrome (pSS). This brief report describes patients identified with a positive SSA antibody and diagnosis of sIBM at a large academic medical center over a 13.5-year period. A cohort identification tool was used to identify patients with positive SSA antibody and a diagnosis of sIBM between January 1, 2006 and June 1, 2019. All cases of sIBM had diagnostic confirmation by a neuromuscular specialist. Demographics, clinical features, autoantibodies, MRI and EMG findings, and muscle biopsy features were reviewed for each identified case. Eight patients were found to carry the diagnosis of pSS and sIBM. Two additional sIBM patients were SSA antibody positive without other pSS features. The mean time from initial symptom onset of muscle weakness to diagnosis was 5.4 years (range 1-15 years). All patients had alternative diagnoses offered for their myopathic symptoms prior to sIBM identification. The NT5c1A antibody was positive in 7 of 8 patients tested. No patient had a durable response to immunosuppressive therapy. The diagnosis of sIBM went unrecognized for over 5 years in our cohort of SSA antibody-positive patients with myopathy. The patients in this cohort were treated with a variety of immunosuppressive agents prior to diagnosis without benefit. Recognizing the clinical features of sIBM in patients with pSS is crucial in instituting appropriate therapy and avoiding unnecessary immunosuppression. Key Points • Sporadic inclusion body myositis (sIBM) can be associated with Sjogren's syndrome. • In this case series, prevalence of the NT5c1A antibody was higher among patients with associated Sjogren's syndrome compared to the cited prevalence of the NT5c1A antibody in patients with isolated sIBM. • It is crucial to consider sIBM in patients with Sjogren's syndrome presenting with motor weakness in order to avoid unnecessary immunosuppression and institute appropriate therapy., (© 2021. International League of Associations for Rheumatology (ILAR).)

Laparoscopic surgery for paraesophageal hernia is well accepted. However, the complications of this relatively new procedure have not been thoroughly investigated. Only four cases of recurrent volvulus after paraesophageal hernia repair have been reported. A 52-year-old man presented with a large right-side paraesophageal hernia. He experienced a retroperitoneal midgastric volvulus despite correct orientation of the stomach distally and proximally. We report an unusual complication that seems congenital in origin. Diagnostic and corrective measures are suggested.

Morgagni hernias are unusual diaphragmatic hernias which usually present in adulthood. They have traditionally been repaired through transabdominal or transthoracic approaches. The authors present a case of a laparoscopic repair of a Morgagni hernia in a 52-year-old female. A tension free repair of the defect was accomplished utilizing Goretex (W.L. Gore & Associates, Inc., North Elkton, MD) mesh. The patient had an uneventful recovery and is asymptomatic at 6 months follow-up. The etiology, diagnosis and traditional surgical approaches to this problem are discussed. A technique for laparoscopic repair of a Morgagni hernia is described. The literature on the laparoscopic repair of a Morgagni hernia is reviewed and different operative techniques are discussed.