Clin Rheumatol (2011) 30:1251–1256 DOI 10.1007/s10067-011-1781-7 BRIEF REPORT Laryngeal involvement in juvenile idiopathic arthritis patients Mosaad Abdel-Aziz & Noha A. Azab & Iman H. Bassyouni & Gehan Hamdy Received: 6 April 2011 /Accepted: 11 May 2011 /Published online: 26 May 2011 # Clinical Rheumatology 2011 Abstract Juvenile idiopathic arthritis (JIA) is an autoim- to be subjected to thorough otolaryngologic examination mune diseases characterized by chronic arthritis and for early diagnosis and prompt management. systemic manifestations. Autoimmune diseases can affect the upper airways including the larynx. The aim of this Keywords Cricoarytenoiditis . Flexible laryngoscopy. study was to investigate laryngeal involvement in JIA Juvenile idiopathic arthritis . Rheumatoid nodule patients and its possible association with JIA disease parameters. Fifty consecutive JIA patients were screened for laryngeal abnormalities using flexible fiberoptic laryn- Introduction goscope and laryngeal computerized tomography. Laryn- geal abnormalities were detected in nine (18%) of our Juvenile idiopathic arthritis (JIA) represents a heteroge- cases, with cricoarytenoiditis in six cases (12%) and a neous group of autoimmune diseases characterized by rheumatoid nodule in the pyriform fossa in only one case chronic arthritis and systemic manifestations [1]. Different (2%). Diffuse congestion and edema of the posterior part of classification criteria have been proposed. The most recent the larynx with normal vocal cord mobility was detected in are those of the International League of Associations for two cases (4%). In our study, laryngeal abnormalities were Rheumatology (ILAR) criteria. The ILAR classification of significantly higher in patients with polyarticular seropos- JIA includes seven subtypes, recognized based on the itive disease subtype and also were significantly higher in clinical features during the first 6 months of disease: patients with longer disease duration, higher disease systemic onset JIA, oligoarticular, polyarticular rheumatoid activity scores, and those with erosive disease. JIA may factor (RF)-positive and RF-negative, enthesitis-related JIA, affect the larynx. Laryngeal involvement in JIA patients is juvenile psoriatic arthritis, and “other arthritides” [2, 3]. more in polyarticular seropositive cases. JIA patients have Laryngeal involvement may occur in autoimmune dis- eases. In rheumatoid arthritis (RA), it may involve the cricoarytenoid joint (CAJ), also it may cause rheumatoid M. Abdel-Aziz nodules on the vocal cords and amyloidosis [4]. CAJ is a Department of Otolaryngology, Faculty of Medicine, diarthrodial synovial joint, in the posterior wall of the Cairo University, Cairo, Egypt larynx, and is important for respiration and phonation [5]. : CAJ arthritis is associated with hoarseness, a sense of N. A. Azab (*) I. H. Bassyouni pharyngeal fullness in the throat on speaking and swallow- Department of Rheumatology and Rehabilitation, ing, referred ear pain and dyspnea [6]. In systemic lupus Faculty of Medicine, Cairo University, Cairo, Egypt erythematosus, laryngeal involvement may occur in the e-mail: [email protected] form of epiglottitis, CAJ arthritis, vocal cord paralysis, mucosal ulceration and hemorrhagic bullae, diffuse edema, G. Hamdy chronic hyperplastic laryngitis, and laryngitis sicca [7]. Department of Internal Medicine, Faculty of Medicine, Cairo University, Moreover, cricoarytenoiditis was reported to occur as the Cairo, Egypt sole presentation during the disease flare [8]. 1252 Clin Rheumatol (2011) 30:1251–1256 JIA has been reported to cause CAJ arthritis leading to Statistical method vocal cord fixation either unilaterally resulting in hoarse- ness of voice or bilaterally resulting in stridor with The data were coded and entered using statistical package difficulty of breathing [9, 10]. Affection of the vocal fold SPSS version 15 for windows. Data were summarized itself has been reported as well [11]. Schwemmle and Ptok using mean±SD for quantitative variables and frequency [12] reported nodes histologically resembling rheumatoid and percentage for qualitative variables. Significant differ- nodules called bamboo nodes. It was the aim of this study ences were calculated using Mann–Whitney U test for to investigate laryngeal involvement in patients with JIA continuous variables. For comparing categorical data, chi- and its possible association with JIA disease parameters. square (χ2) test with Yates" correction or Fisher"s exact tests were used. P value<0.05 was considered significant. Patients and methods Results Fifty consecutive patients with JIA diagnosed according to the ILAR criteria [2] were included in the present study. All Fifty consecutive JIA patients were included in the present patients had their onset of the disease before the age of study; their clinical and demographic data are shown in 16 years, with arthritis lasting for at least 6 weeks" duration Table 1. Extra-articular involvement was found in five and with other identifiable causes of arthritis excluded [2]. All cases were attending and were recruited from the Table 1 Clinical, demographic, and laboratory features of JIA N Rheumatology and Rehabilitation Department and Internal patients ( =50) Medicine Department, Faculty of Medicine, Cairo Univer- Females N (%) 34 (68) sity in the period from March 2008 to April 2010. The Males N (%) 16 (32) protocol for this research conforms to the provisions of the Age (years) (mean±SD) 13.6±4.64 " World Medical Association s Declaration of Helsinki and Age at onset (years) (mean±SD) 8.12±3.5 informed consent has been obtained from all participants Disease duration (years) (mean±SD) 5.56±3.5 and/or their parents before the study. The study was JIA subtypes performed after institutional board approval. Polyarticular JIA RF+ n (%) 8(16) All patients were subjected to full history taking and Polyarticular JIA RF- n (%) 7(14) thorough clinical examination. Also, routine laboratory Oligoarticular JIA n (%) 26(52) tests were conducted at the day of examination in the form Persistent type n (%) 9 (18) of complete blood picture, erythrocyte sedimentation rate, Extended type n (%) 17(34) C-reactive protein, rheumatoid factor by latex agglutination Systemic onset JIA n (%) 7(14) test, antinuclear antibodies, liver and kidney functions, and Enthesitis-related JIA n (%) 2 (4) complete urine analysis. Psoriatic JIA n (%) 0 (0) All patients underwent clinical evaluation of disease others n (%) 0 (0) activity as assessed by the disease activity score, using a Extra-articular manifestations n (%) 5(10) 28-joint score (DAS-28) [13]. Postero-anterior radiographs Micrognathia n (%) 15(30) of hands, wrists, and forefeet were obtained at inclusion in Medications (in the past 3 months) the study, and joint destruction was classified by compar- n ison with standard reference films according to the Larsen- Methotrexate (%) 36 (72) n Dale index [14]. The patient was considered to have an Antimalarials (%) 24 (48) n erosive disease on finding of at least one definite erosion on Corticosteroids (%) 14 (28) n any of the hands or feet radiographs. Lefulonamide (%) 4 (8) History of coughing, change in voice, breathing diffi- DAS28 (mean±SD) 6.2±1.34 culties, or chronic dysphagia was thoroughly investigated. ESR mm/h (mean±SD) 29.2±25.1 Laryngeal examination was done using flexible laryngos- CRP mg/dl (mean±SD) 6.3±2.4 copy to detect CAJ inflammation or ankylosis, mobility of RF (+ve) n (%) 23(46) the vocal cords, or the presence of laryngeal rheumatoid ANA (+ve) n (%) 11 (22) nodules. Also, CT of the larynx was done to all patients to Anti CCP n (%) 6(12) detect CAJ synovial thickening or erosions, arytenoid Erosive arthritis n (%) 18 (36) subluxation, asymmetry of the glottis or aryepiglottic folds, DAS 28 disease activity score, ESR erythrocyte sedimentation rate, or rheumatoid nodules. CT scans were done during quite CRP C-reactive protein, RF rheumatoid factor, ANA antinuclear respiration, with 2-mm thickness scan sections. antibodies, anti CCP anti-cyclic citrullinated peptide Clin Rheumatol (2011) 30:1251–1256 1253 cases (10%) mainly in the form of pericardial effusion. Micrognathia was found in 15 cases (30%) which was a potential cause of difficult intubation during laryngoscopy. As regards symptoms of laryngeal involvement, only four cases (8%) complained of change of voice; two of them (4%) gave history of chronic dysphagia, and the other two cases (4%) complained of unilateral throat pain with referred earache. Also, two of our patients (4%) complained of breathing difficulty following severe upper respiratory tract infection and were managed by tracheostomy after failure of conservative measures which included antibiotics and steroids. Fig. 1 The larynx as seen by flexible laryngoscopy, the arrow points Flexible laryngoscopy showed laryngeal abnormalities in to a nodule in the left pyriform fossa nine patients (18%); two cases (4%) showed unilateral vocal cord immobility on the right side with complete compensation of chink by the left cord, two cases(4%) cases (18%). Laryngeal involvement was in the form of showed congestion, and edema of one arytenoid with bilateral vocal cord paramedian location in two cases (4%) sluggish mobility of the related vocal cord (of the right in indicating alteration of cricoarytenoid motion that limited one case and of the left in the other), two cases(4%) showed full lateral rotation; vocal cord thickening in three cases congestion and edema of both arytenoids