Voice disorders in Sjögren's syndrome: Prevalence and related risk factors.

OBJECTIVES/HYPOTHESIS: Sjögren's Syndrome (SS) is an autoimmune disease that causes sicca (dryness) symptoms by affecting secretions most notably of the lacrimal and salivary glands. Voice disorders have been documented in patients with SS, but the true prevalence and relationships among possible contributing factors remain unknown. This preliminary epidemiological investigation examined prevalence and risk factors for voice disorders in SS. STUDY DESIGN: Self-report epidemiological questionnaire. METHODS: One hundred and one (101) patients with SS (94 females, 7 males; M age = 59.4 years; standard deviation [SD] = 14.1 years) completed an extensive interview using a previously validated questionnaire involving the patient's medical, family, occupational, psychosocial, social/lifestyle, voice use, and general health histories. Summary statistics, chi-squares, risk ratios, and multiple logistic regression were used to determine the frequency and severity of voice disorders in individuals with SS, as well as associations with demographic, lifestyle, health, disease severity, and voice use factors. RESULTS: The prevalence of a current voice disorder in individuals with SS was 59.4%. In general, voice disorders began gradually; were chronic; and correlated with SS disease severity independent of age, sex, duration of the disease, comorbid autoimmune conditions, and use of SS-related medication. Specific voice symptoms including chronic throat dryness and soreness were significantly associated with SS disease severity. CONCLUSIONS: Voice disorders are relatively common in SS and are more frequent as disease severity worsens. These findings have important implications for evaluation and treatment of patients with SS. LEVEL OF EVIDENCE: 4.

TGFß and PDGF-B signaling blockade inhibits myofibroblast development from both bone marrow-derived and keratocyte-derived precursor cells in vivo.

Myofibroblasts, the primary cells associated with corneal stromal haze (opacity), can be derived from both cornea-derived and bone marrow-derived precursor cells. In the present study, the role of TGFß or PDGF blockage on bone marrow-derived myofibroblast development was investigated using a green fluorescent protein (GFP) chimeric bone marrow mouse model and plasmid vectors that blocked TGFß or PDGF signaling. At the peak of corneal haze one month after irregular phototherapeutic keratectomy the central stroma had significantly less alpha-smooth muscle actin (α-SMA)-positive cells derived from GFP+ bone marrow-derived cells or GFP- keratocyte/corneal fibroblast-derived cells when corneas were treated with the TGFß blocking vector pGFPC1.TGFRBKDEL or the PDGF blocking vector pCMV.PDGFRB.23KDEL compared with the corresponding empty vector treated or untreated control groups. In individual animals, 30-60% of myofibroblasts were derived from bone marrow-derived precursor cells and 40-70% of myofibroblasts were derived from keratocyte-derived precursor cells. TGFß and PDGF regulate corneal myofibroblast development from bone marrow-derived precursor cells and keratocyte/corneal fibroblast-derived precursor cells.