Audiovestibular manifestations in patients with limited systemic sclerosis and centromere protein-B (CENP-B) antibodies.

Audiovestibular dysfunction has been reported in patients with connective tissue disease. Systemic sclerosis (SSc; scleroderma) is a rare connective tissue disease of unknown etiology. In the current study we assess whether audiovestibular involvement is present in patients with limited scleroderma (lSSc). To answer this question we studied a series of 35 consecutive patients who fulfilled well-established classification criteria for lSSc and had antibodies against the major centromere protein-B (CENP-B), and 59 matched controls. Individuals with a history of cerebrovascular complications, syphilis, Ménière and other vestibular syndromes, infections involving the inner ear, barotrauma, or in treatment with ototoxic drugs were excluded. The majority of patients with lSSc were women (94%). The mean age at time of study was 64.5 years, and the mean age at time of disease diagnosis was 56.9 years. Besides Raynaud phenomenon, most patients with lSSc had other typical features of CREST (calcinosis, Raynaud phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia) syndrome. Twenty-seven (77%) patients showed abnormal hearing loss in the audiogram compared with only 15 (26%) of the controls (p < 0.001). Values of audiometric tests (pure-tone average and speech reception threshold) yielded significant differences between patients and controls (p < 0.001). The typical pattern of hearing impairment in our series of lSSc patients was a bilateral and symmetrical sensorineural hearing loss with a flat pattern in the audiogram. Abnormal tympanogram and abnormal stapedial reflex were more commonly observed in patients than controls (p < or = 0.001). Similarly, a significantly increased frequency of abnormal oculocephalic response (10 patients, 29%) and head-shaking nystagmus (9 patients, 26%) was observed in patients compared with controls (p < 0.001 for both comparisons). Finally, a significantly increased frequency of abnormal caloric test and clinical test of sensory integration and balance was observed in lSSc patients (31% and 46% of patients, respectively) compared with controls (0% and 12%, respectively) (p < 0.001 for both comparisons). The current study demonstrates strong evidence for inner ear compromise in patients with lSSc.

Benign paroxysmal positional vertigo and clinical test of sensory interaction and balance in systemic sclerosis.

OBJECTIVE: To assess the frequency and characteristics of benign paroxysmal positional vertigo (BPPV) and clinical test of sensory interaction and balance (CTSIB) abnormalities in patients with systemic sclerosis (SSc). STUDY DESIGN: A series of consecutive patients diagnosed with SSc according to well-established classification criteria and matched controls were studied. SETTING: The study was performed at the otolaryngology division of a tertiary reference center. PATIENTS: Forty-two patients (35 with limited SSc [lSSc] and 7 with diffuse SSc [dSSc]) and 74 controls were studied between January and May 2007. INTERVENTION: Dix-Hallpike and cephalic rotational tests and CTSIB were performed in SSc patients and age-, sex-, and ethnically frequency-matched controls. MAIN OUTCOME MEASURE: Type and frequency of BPPV and CTSIB conditions were assessed. RESULTS: Seven patients (17%) fulfilled the diagnostic criteria for BPPV compared with none of the controls (p < 0.001). It was related to the involvement of the posterior semicircular canal in two lSSc patients and the horizontal semicircular canal in another three patients with lSSc and two with dSSc. A significantly increased frequency of abnormal CTSIB was also observed in SSc patients (20 [48%]) compared to controls (7 [10%]; p < 0.0001; odds ratio, 8.70; 95% confidence interval, 2.97-27.2). It was caused by a vestibular pattern in most patients (p < 0.0001). CONCLUSION: The present study shows an increased frequency of BPPV and a vestibular pattern in CTSIB in SSc patients.

Systemic sclerosis in northwestern Spain: a 19-year epidemiologic study.

To investigate the epidemiology of systemic sclerosis (SSc) in southern Europe, we assessed the incidence, prevalence, clinical spectrum, and survival of patients diagnosed with SSc in the Lugo region of northwestern Spain. Between January 1988 and December 2006, SSc was diagnosed in 78 Lugo residents according to the criteria proposed by LeRoy and Medsger and/or the 1980 American College of Rheumatology (ACR) preliminary criteria for the classification of SSc. However, only 44 (56.4%) of the 78 patients fulfilled the 1980 ACR criteria for the classification of SSc. The mean age at the time of disease diagnosis was 59.8 +/- 13.3 years. Twenty-three (29.5%) met definitions for diffuse SSc (dSSc), and 55 (70.5%) for limited SSc (lSSc). Patients with lSSc had a longer disease duration before the diagnosis (10.2 +/- 12.0 yr) than those with dSSc (3.7 +/- 3.2yr) (p < 0.001). Based on the criteria proposed by LeRoy and Medsger and/or the 1980 ACR criteria for the classification of SSc, the overall age- and sex-adjusted annual incidence rate over the 19-year study period was 2.3 (95% confidence interval [CI], 1.6-2.5) per 100,000 population aged 15 yr and older (women: 3.5 [95% CI, 2.3-3.9]; men: 1.0 [95% CI, 0.5-1.4]; p < 0.001). Using only the 1980 ACR criteria for SSc, the total annual-adjusted incidence rate was 1.2 (95% CI, 0.9-1.6) per 100,000 population aged 15 years and older (women: 1.8 [95% CI, 1.2-2.5]; men: 0.7 [95% CI, 0.3-1.2]; p < 0.001). The incidence increased significantly in individuals aged 45 years or older. The overall incidence rates of SSc increased over the length of the study (p for trend in the total incidence < 0.001). This was mainly due to a progressive increase of SSc in women between 1993 and 2002. By December 31, 2006, the overall age-adjusted SSc prevalence in the Lugo region of patients who met the criteria proposed by LeRoy and Medsger and/or the 1980 ACR criteria was 27.7 (95% CI, 21.1-35.84) per 100,000 population aged 15 years and older. Cardiopulmonary complications were the leading cause of death (13 of 20 cases). Compared with that in the general population, the probability of survival in patients with SSc was significantly reduced (p < 0.001).The current study establishes a baseline estimate of the incidence and clinical spectrum of SSc in northwestern Spain. According to our results, the incidence and prevalence of SSc in northwestern Spain are similar to those found in Greece and some regions of the United States. Our data confirm a reduced probability of survival in patients with SSc.