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Pemphigus, an autoimmune blistering disease that affects the skin and mucous membranes, adversely impacts patients' quality of life (QOL). While there are various QOL measurement tools that can be used in this disease, few studies have assessed how a patient's change in disease severity can affect their QOL. This study aims to identify which disease severity index correlates best with the change in QOL. Fifty pemphigus patients completed QOL surveys with disease severity scored over two visits. QOL was assessed with the Autoimmune Bullous Disease Quality of Life (ABQOL), Dermatology Life Quality Index (DLQI), Skindex-29, and Short Form Survey 36 (SF-36). Disease severity was scored with the Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). Correlations between the change in QOL scores and change in disease severity were analyzed using Spearman's coefficient (r). The change in PDAI showed a strong correlation (r = 0.60-0.79) with changes in the ABQOL, Skindex-29 symptoms (Skindex-S), and Skindex-29 functioning (Skindex-F) subscales for all patients (n = 50). For patients with mucosal disease (n = 24), the change in PDAI showed a strong correlation with changes in the ABQOL and Skindex-S subscale. For patients without mucosal disease, the change in PDAI showed a strong correlation with the Skindex-S. The change in ABSIS showed a strong correlation with Skindex-S for all patients and patients with no mucosal involvement, but showed no strong correlations for patients with mucosal involvement. The changes in PDAI always had a stronger correlation than the changes in ABSIS scores to changes in the ABQOL, DLQI, and Skindex-29 subscales, except where the PDAI and ABSIS scores were about the same for the Skindex-S subscale in patients with no mucosal involvement (r = 0.76 and r = 0.77, respectively). PDAI is superior to ABSIS in its correlation with validated QOL tools. The QOL tools that appear to be of most use in clinical trials and patient management are the Skindex-S and ABQOL.

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OBJECTIVE: Systemic lupus erythematosus (SLE) is a disorder that is heterogeneous and can be difficult to diagnose. One hallmark of the disease is the presence of antinuclear antibodies (ANAs), a feature that has been incorporated into multiple classification criteria over the years. In this study, we used a database of patients with cutaneous lupus erythematosus (CLE) to determine how many had a negative ANA and met criteria for SLE using the American College of Rheumatology (ACR) and/or Systemic Lupus International Collaborating Clinics (SLICC) criteria. METHODS: We used a database of 301 biopsy-proven CLE patients that contained information including ANA status and the presence of features of SLE. The database was searched for patients who had a negative ANA result and whether or not they met SLE criteria using the ACR and/or SLICC criteria. RESULTS: Of the 301 patients with biopsy-proven CLE and a known ANA, 111 had a negative ANA test (36.9%) and 27 had an ANA test that fluctuated (33.3%). In all, 20 ANA-negative patients met SLE criteria (18.0%), and 12 patients with a fluctuating ANA test met SLE criteria (44.4%). Of all patients who had either a negative or fluctuating ANA result and who met criteria for SLE (n = 32), 27 patients had involvement of ≥1 organ system other than skin (84.4%), and 13 patients had involvement of ≥2 organ systems other than skin (40.6%). CONCLUSION: Our results show that an ANA is not always present in patients with systemic disease. This fact should be taken into consideration when devising SLE classification criteria to be used for clinical trials.

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OBJECTIVE: The objectives were to investigate the function of central auditory pathways and of the medial efferent olivocochlear system (MOCS). DESIGN: Event-related potentials (ERP) were recorded following the delivery of the stimulus /da/ in quiet and in ipsilateral, contralateral, and binaural noise conditions and correlated to the results of the auditory processing disorders (APD) diagnostic test battery. MOCS function was investigated by adding ipsilateral, contralateral, and binaural noise to transient evoked otoacoustic emission recordings. Auditory brainstem responses and pure tone audiogram were also evaluated. STUDY SAMPLE: Nineteen children (7 to 12 years old) with APD were compared with 24 age-matched controls. RESULTS: Otoacoustic emissions and ABR characteristics did not differ between groups, whereas ERP latencies were significantly longer and of higher amplitudes in APD children than in controls, in both quiet and noise conditions. The MOCS suppression was higher in APD children. CONCLUSIONS: Findings indicate that children with APD present with neural deficiencies in both challenging and nonchallenging environments with an increase in the timing of several central auditory processes correlated to their behavioural performances. Meanwhile, their modulation of the auditory periphery under noisy conditions differs from control children with higher suppression.

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Spontaneous otoacoustic emissions (SOAEs) are found in most healthy ears, and can be used to measure the health of the cochlear structures and feedback mechanism. According to existing literature, right ears tend to exhibit greater numbers of SOAEs than left ears (Bilger et al., 1990) and females tend to show higher incidence of SOAEs than males (Moulin et al., 1993). The SOAE prevalence has not been extensively studied in children with Auditory Processing Disorder (APD), a disorder with unknown etiology that reduces one's ability to process auditory information. This study examined the prevalence and ear advantage of SOAEs between genders in children diagnosed with APD. SOAEs were investigated in 19 children (7 girls and 12 boys) with APD and 2 4 typically developing children (14 girls and 10 boys) aged 7-12. Right ear advantage was more prevalent in control (71%) than APD subjects (42 %). However, over 30% more females exhibited a right ear advantage than males in each group. Although the results are not significant, our findings indicate that the lack of right ear advantage for SOAE is more prevalent in children with APD, particularly in males, suggesting that cochlear mechanisms or their control might be somehow affected in APD.