Hospital Universitario Marqués de Valdecilla Anero, Spain
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Carmen Lasa1, Raul Fernandez-Ramon2, Jorge Javier Gaitán2, Jose Luis Martin-Varillas3, Rosalía Demetrio2, Ivan Ferraz Amaro4, Santos Castañeda5 and Ricardo Blanco6, 1Hospital Universitario Marqués de Valdecilla, IDIVAL., La Cavada, Spain, 2Hospital Universitario Marqués de Valdecilla, Santander, Spain, 3Hospital de Laredo, Laredo, Spain, 4Hospital Universitario de Canarias, Santa Cruz de Tenerife, Spain, 5Hospital de la Princesa, Madrid, Spain, 6Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
Background/Purpose: Sarcoidosis is a disease with heterogeneous clinical presentation and course. The objective of this study is the identification of clinical phenotypes using cluster analysis.
Methods: A model-based clustering relaying on 19 clinical variables was performed in a retrospective cohort of 342 sarcoidosis patients, diagnosed and follow-up from 1999 to 2019 in a hospital at Northern Spain. Chi-square test and ANOVA were used to compare categorical and continuous variables among groups. Two-sample t-tests and the partition of Pearson's chi-square statistic were used in pairwise comparisons. The Wasfi severity score was calculated and compared among clusters.
Results: Cluster analysis identified five groups: C1: erythema nodosum and articular involvement (n=55; 16.1%), C2: miscellaneous extrapulmonary sarcoidosis (n=49; 14.3%), C3: ocular and/or neurological involvement (n=83; 24.3%), C4: isolated hiliar adenopathy (n=17; 5.0%), and C5: parenchymal lung involvement with dyspnea (n=138; 40.4%). Lung involvement was predominant in all clusters, ranging from 89.9% (C5) to 100% (C1 and C4), except for C2 (55.1%). Extrapulmonary involvement was significantly higher in C2 (96.4%) and C3 (98.0%). Demographic and clinical characteristics are shown in Table 1. A significant low mean FEV1 was detected in C5 (90.5±21.8) versus C1 (102.0±22.9), C3 (102.3±17.6) and C4 (105.8±20.8). The cluster 5 had a significantly lower mean FVC (96.6±18.9) than the other clusters, ranging from 108.1±18.0 (C3) to 111.5±21.7 (C4). The prescription of systemic steroids and non-corticosteroid immunosuppressants was significantly higher in the clusters 1, 3 and 5. Chronicity rates were significantly higher in C3 (31.3%) and C5 (32.6%) compared to C1 (9.1%) and C4 (0%). The clusters 3 and 5 also showed significantly higher severity score values. According to the clusters identified in the present study, we proposed an individualized "treat to target" schedule by subgroups (Figure 1).
Conclusion: Five phenotypes of sarcoidosis with different clinical and prognostic characteristics are proposed in our study. Cluster analysis can be a useful tool for identifying clinical patterns in a disease as heterogeneous as sarcoidosis and facilitating its management.
C. Lasa: None; R. Fernandez-Ramon: None; J. Gaitán: None; J. Martin-Varillas: None; R. Demetrio: None; I. Ferraz Amaro: AbbVie/Abbott, 5, 6, Amgen, 5, 6, Bristol-Myers Squibb(BMS), 6; S. Castañeda: None; R. Blanco: AbbVie, 5, 6, Amgen, 6, AstraZeneca, 2, BMS, 6, Eli Lilly, 6, Galapagos, 2, 6, Janssen, 2, 6, MSD, 6, Novartis, 2, 6, Pfizer, 2, 6, Roche, 5, 6, Sanofi, 6.