University of Connecticut Health Center Farmington, CT
Introduction: Lichen planus (LP) is an inflammatory skin condition that affects oropharyngeal and esophageal mucosa. Esophageal LP (ELP) has a prevalence of 0.19% but is thought to be underdiagnosed. It presents with ulceration, exudates, rings and/or strictures. Although pathogenesis of LP is incompletely understood, the JAK signaling pathway is thought to play a role. Upadacitinib is a selective JAK1 inhibitor approved for use in ulcerative colitis. We present a case of refractory ELP with endoscopic and histologic response to upadacitinib after failing standard treatment.
Case Description/Methods: A 68 year old female with history of Sjogren’s syndrome, LP, and prior peptic stricture at the gastroesophageal junction presented with dysphagia to both liquids and solids, intermittent reflux, regurgitation, and self-limited impactions. An EGD showed esophageal stenosis, severe esophagitis, and mucosal ulceration (Fig. 1a). Passage of the scope resulted in dilation; however, formal dilation of stenosis was not performed due to severity of esophagitis. Esophageal biopsies revealed squamous mucosa with parakeratosis, dyskeratotic cells and scattered lymphocytes consistent with ELP. Subsequently, the patient recalled having a remote history of oral lichen planus. She was started on pantoprazole 40 mg twice daily and budesonide oral slurry with limited response. With co-management by gastroenterology and dermatology, she was initiated on mycophenolate 500 mg daily and increased to 2000 mg twice a day. She continued to lack both histologic and symptomatic improvement remaining limited to a soft and liquid diet resulting in a 20lb weight loss. She was started on monotherapy with upadacitinib 30 mg twice daily. This resulted in marked symptomatic recovery with repeat endoscopy 3 months later showing dramatic macroscopic and histologic improvement (Fig. 1b), with only some residual fibrostenotic disease remaining (Fig. 1c).
Discussion: Multimodal treatment is typically employed in ELP with medical therapy and endoscopic dilation. Systemic or topical steroids are first line, with efficacy estimated at 60-70%. Other therapies include tacrolimus, cyclosporine, mycophenolate, rituximab, adalimumab, and tofacitinib, however data for these therapies are limited. To date, there are only two cases reported in the literature of successful treatment of oro-esophageal LP with upadacitinib. Given profound improvement seen in our patient, upadacitinib may offer another alternative for these difficult-to-treat patients.
