Highlights
- •We identified six phenotype subgroups
- •Löfgren's syndrome comprised 3 clusters (C1, C2, and C3)
- •C4 cluster comprised patients with pulmonary sarcoidosis
- •C5 cluster comprised patients with abdominal and pulmonary sarcoidosis
- •C6 cluster comprised patients with stage I plus extrapulmonary sarcoidosis
Abstract
Background
Sarcoidosis is a heterogeneous disease with high variability in natural history and
clinical spectrum. The study aimed to reveal different clinical phenotypes of patients
with similar characteristics and prognosis.
Methods
Cluster analysis including 26 phenotypic variables was performed in a large cohort
of 694 sarcoidosis patients, collected and followed-up from 1976 to 2018 at Bellvitge
University Hospital, Barcelona, Spain.
Results
Six homogeneous groups were identified after cluster analysis: C1 (n=47; 6.8%), C2
(n=85; 12.2%), C3 (n=153; 22%), C4 (n=29; 4.2%), C5 (n=168; 24.2%), and C6 (n=212;
30.5%). Presence of bilateral hilar lymphadenopathy (BHL) ranged from 65.5% (C4) to
97.9% (C1). Patients with Löfgren syndrome (LS) were distributed across 3 phenotypes
(C1, C2, and C3). In contrast, phenotypes with pulmonary (PS) and/or extrapulmonary
sarcoidosis (EPS) were represented by groups C4 (PS 100% with no EPS), C5 (PS 88.7%
plus EPS), and C6 (EPS). EPS was concentrated in groups C5 (skin lesions, peripheral
and abdominal lymph nodes, and hepatosplenic involvement) and C6 (skin lesions, peripheral
lymph nodes, and neurological and ocular involvement). Unlike patients from LS groups,
most patients with PS and/or EPS were treated with immunosuppressive therapy, and
evolved to chronicity in higher proportion. Finally, the cluster model worked moderately
well as a predictive model of chronicity (AUC=0.705).
Conclusion
Cluster analysis identified 6 different clinical patterns with similar phenotypic
variables and predicted chronicity in our large cohort of patients with sarcoidosis.
Classification of sarcoidosis into phenotypes with prognostic value may help physicians
to improve the efficacy of clinical decisions.
Keywords
Abbreviations:
AA (arthralgias/arthritis), AUC (area under curve), BHL (bilateral hilar lymphadenopathy), COS (clinical outcome status), CPI (composite physiology index), DLco (diffusing capacity for carbon monoxide), EN (erythema nodosum), EPS (extrapulmonary sarcoidosis), FEV1 (forced expiratory volume in 1st second), FVC (forced vital capacity), LS (Löfgren's syndrome), MPAD/AAD (main pulmonary artery diameter/ascending aorta diameter ratio), PAI (periarticular ankle inflammation), PS (pulmonary sarcoidosis), ROC (receiver operating characteristic), SACE (serum angiotensin-converting enzyme), SURT (sarcoidosis of the upper respiratory tract)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: April 21, 2020
Accepted:
April 6,
2020
Received:
October 11,
2019
Identification
Copyright
© 2020 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
