Adult-onset Still's Disease with Dysphagia: Case report

ABSTRACT

We experienced a case of adult-onset Still's disease with dysphagia of soft tissue origin. A 38-year-old woman was admitted for intermittent high spiking fever, diffuse pain and swelling on anterior neck with dysphagia. Physical examination revealed a thin woman with trismus, board-like hardness of anterior neck, hepatomegaly, and erythematous evanescent rash on leg. Neck CT displayed bilateral cervical lymphadenopathy with soft tissue swelling, and tonsilitis. Chest and abdomen CT showed the inflammatory changes of multiple organs. Laboratory evaluation revealed neutrophilic leukocytosis, slightly increased alanine transaminase, negative antinuclear antibody and rheumatoid factor, and increased inflammatory markers. Skin, liver, colon, and pleural biopsy demonstrated the chronic inflammation. Videofluoroscopic swallowing study revealed laryngeal penetration with large amount residue in vallecular space and pyriform sinus, and incomplete cricopharyngeal relaxation. Gradual improvement in anterior neck pain and pulmonary edema and the decrease of effortful and multiple swallowing were observed with high dose steroid therapy.