akamatsu konomi

OBJECTIVES: To investigate the prevalence of aspiration and its association with GI involvement and clinical features in systemic sclerosis (SSc). METHODS: Fifty patients with SSc who underwent the videofluoroscopic (VF) swallowing study were included. Aspiration was identified by the presence of aspiration and/or penetration defined by the Penetration-Aspiration Scale. The radiological findings including the residue in the oral cavity, pharynx and esophagus, which reflect oral, pharyngeal, and esophageal involvement, were also evaluated. RESULTS: Twenty-three patients (46%) had aspiration and/or penetration. Patients with aspiration and/or laryngeal penetration had more pharyngeal vallecular residue (None 22%, Trace 44%, Mild 56%; p = 0.072) and esophageal residue (None 0%, Mild 20%, Moderate 40%, Severe 61%; p = 0.010). Esophageal, pharyngeal, and lower GI involvement were found in 98%, 83%, and 54%, respectively. Notably, the group with esophageal and pharyngeal plus lower GI involvement had a higher frequency of aspiration and/or penetration (79% vs 28%; p = 0.003), higher FSSG scores (18.4 ± 11.8 vs 5.4 ± 5.7; p = 0.002), and higher UCLA GIT-2.0 total scores (None-to-Mild 47% vs 100%, Moderate 42% vs 0%, Severe-to-Very severe 11% vs 0%; p = 0.001) compared to those limited esophageal and pharyngeal involvement. By multivariate analysis, aspiration and/or penetration was associated with pharyngeal vallecular residue (Odds ratio (OR) 3.71; p = 0.012) and esophageal residue (OR 2.92; p = 0.011), and higher UCLA GIT-2.0 scores for diarrhea (OR 3.68; p = 0.028) and the total score (OR 4.21; p = 0.046). CONCLUSION: In our study, about half of the patients had aspiration and/or penetration in SSc. Aspiration was associated with the extent of radiographic abnormalities of pharyngeal and esophageal involvement on the VF swallowing study. Patients with lower GI have aspiration and high UCLA GIT-2.0 scores suggesting that these patients had more severe GI manifestation.

OBJECTIVE: To update and revise the diagnostic criteria for mixed connective tissue disease (MCTD) issued by the Japan Research Committee of the Ministry of Health, Labor, and Welfare (MHLW), a round table discussion by experts from rheumatology, dermatology, and pediatric medicine was conducted in multiple occasions. METHODS: The definition of MCTD, and items included in the diagnostic criteria were generated by consensus method and evaluation using clinical data of typical and borderline cases of MCTD, by applying to the diagnostic criteria for MCTD proposed in 1996 and 2004 by the Research Committee of MHLW. RESULTS: To the end, all committee members reached consensus. Then, the criteria were assessed in an independent validation cohort and tested against preexisting criteria. The revised criteria facilitate an understanding of the overall picture of this disease by describing the concept of MCTD, common manifestations, immunological manifestation and characteristic organ involvement. Conditions with characteristic organ involvement include pulmonary arterial hypertension, aseptic meningitis and trigeminal neuropathy. Even if the overlapping manifestations are absent, MCTD can be diagnosed based on the presence of the characteristic organ involvement. Furthermore, the criteria were validated for applicability in actual clinical cases, and public comments were solicited from the Japan College of Rheumatology and other associated societies. CONCLUSION: After being reviewed through public comments, the revised diagnostic criteria have been finalized.