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Systemic phenotype of sarcoidosis associated with radiological stages. Analysis of 1230 patients

Published:September 11, 2019DOI:https://doi.org/10.1016/j.ejim.2019.08.025

      Highlights

      • Sarcoidosis at diagnosis was limited to extrathoracic organs in 8% of patients.
      • Skin and neurological involvements were the most common isolated extrathoracic presentations.
      • Patients with Scadding stage I (isolated BHL) represented one third of patients.
      • The presence or absence of BHL played a weak role in driving significant phenotypic differences.
      • Stage IV (radiological evidence of lung fibrosis, 3% of patients) was associated with older age.

      Abstract

      Background

      To analyze the association between Scadding radiological stages of sarcoidosis at diagnosis and the disease phenotype (epidemiology, clinical presentation and extrathoracic involvement) in one of the largest cohorts of patients with sarcoidosis reported from southern Europe.

      Methods

      The SARCOGEAS-Study Group includes a multicenter database of consecutive patients diagnosed with sarcoidosis according to the WASOG 1999 criteria. Extrathoracic disease at diagnosis was defined according to the 2014 instrument and the clusters proposed by Schupp et al.

      Results

      We analyzed 1230 patients (712 female, mean age 47 yrs.) who showed the following Scadding radiologic stages at diagnosis: stage 0 (n = 98), stage I (n = 395), stage II (n = 500), stage III (n = 195) and stage IV (n = 42). Women were overrepresented in patients presenting with extrathoracic/extrapulmonary disease, while the diagnosis was made at younger ages in patients presenting with BHL, and at older ages in those presenting with pulmonary fibrosis (q values <0.05). Multivariable adjusted analysis showed that patients presenting with pulmonary involvement (especially those with stages II and III) had a lower frequency of concomitant systemic involvement in some specific extrathoracic clusters (cutaneous-adenopathic/musculoskeletal, ENT and neuro-ocular/OCCC) but a higher frequency for others (hepatosplenic), in comparison with patients with extrapulmonary involvement (stages 0 and I). The presence of either BHL or fibrotic lesions did not influence the systemic phenotype of patients with pulmonary involvement.

      Conclusions

      The key determinant associated with a differentiated systemic phenotype of sarcoidosis at diagnosis was interstitial pulmonary involvement rather than the individual Scadding radiological stage.

      Abbreviations:

      WASOG (World Association of Sarcoidosis and Other Granulomatous Disorders), BHL (Bilateral hilar adenopathies), PI (Pulmonary infiltrates), ENT (ear, nose, and throat), CT (computed tomography), OCCC (oculo-cardiac-central nervous system-cutaneous)

      Keywords

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