J Korean Rheum Assoc.  2006 Sep;13(3):242-246.

A Case of Laryngeal Involvement as the Sole Presenting Manifestation of Systemic Lupus Erythematosus

• Lee YA
• Jeong SG
• Kim SH
• Nam GY
• Park JH

Abstract

Laryngeal involvement is a rare complication of systemic lupus erythematosus (SLE) and can range from mild mucosal inflammation, vocal cord paralysis, and edema to necrotizing vasculitis with airway obstruction. Cricoarytenoid arthropathy is rare cause of severe airway obstruction in laryngeal involvement of SLE. In contrast to patients with rheumatoid arthritis, patients with SLE typically present with acute arthritis of cricoarytenoid joints and respond to corticosteroid therapy alone. We describe here a 13-year-old girl with SLE who presented with intermittent fever, swallowing difficulty, vocal cord paralysis and acute dyspnea required emergent tracheostomy as the sole manifestation of active SLE. She was diagnosed as having SLE based on the findings of positive anti-nuclear antibody and anti-ds DNA antibody with high titers, hypocomplementemia, leukocytopenia, and transient polyarthritis and proteinuria. No other clinical symptoms were found. Endoscopic findings showed edema of aryepiglottic fold, bilateral fixation of the vocal cords and edematous supraglottic structure with engorged vessels. Computered tomography scan of the neck showed diffuse soft tissue edema around the arytenoid and cricoid cartilages and enhancement of the aryepiglottic fold.

Keyword

SLE; Larynx; Cricoarytenoiditis

MeSH Terms

Adolescent
Airway Obstruction
Arthritis
Arthritis, Rheumatoid
Cricoid Cartilage
Deglutition
DNA
Dyspnea
Edema
Female
Fever
Humans
Inflammation
Joints
Larynx
Leukopenia
Lupus Erythematosus, Systemic*
Neck
Proteinuria
Tracheostomy
Vasculitis
Vocal Cord Paralysis
Vocal Cords
DNA