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Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis: Cohort study of a 40-year clinical experience at a tertiary referral center in Barcelona, Spain.

Abstract Cohort studies of large series of patients with sarcoidosis over a long period of time are scarce. The aim of this study is to report a 40-year clinical experience of a large series of patients at Bellvitge University Hospital, a tertiary university hospital in Barcelona, Spain. Diagnosis of sarcoidosis required histological confirmation except in certain specific situations. All patients underwent a prospective study protocol. Clinical assessment and follow-up of patients were performed by a multidisciplinary team.From 1976 to 2015, 640 patients were diagnosed with sarcoidosis, 438 of them (68.4%) were female (sex ratio F/M 2:1). The mean age at diagnosis was 43.3 ± 13.8 years (range, 14-86 years), and 613 patients (95.8%) were Caucasian. At diagnosis, 584 patients (91.2%) showed intrathoracic involvement at chest radiograph, and most of the patients had normal pulmonary function. Erythema nodosum (39.8%) and specific cutaneous lesions (20.8%) were the most frequent extrapulmonary manifestations, but there was a wide range of organ involvement. A total of 492 patients (76.8%) had positive histology. Follow-up was carried out in 587 patients (91.7%), over a mean of 112.4 ± 98.3 months (range, 6.4-475 months). Corticosteroid treatment was administered in 255 patients (43.4%), and steroid-sparing agents in 49 patients (7.7%). Outcomes were as follows: 111 patients (18.9%) showed active disease at the time of closing this study, 250 (42.6%) presented spontaneous remission, 61 (10.4%) had remission under treatment, and 165 (28.1%) evolved to chronic sarcoidosis; among them, 115 (19.6%) with mild disease and 50 (8.5%) with moderate to severe organ damage. A multivariate analysis showed that at diagnosis, age more than 40 years, the presence of pulmonary involvement on chest radiograph, splenic involvement, and the need of treatment, was associated with chronic sarcoidosis, whereas Löfgren syndrome and mediastinal lymphadenopathy on chest radiograph were indicators of good outcome.Sarcoidosis is a multisystem disease with protean clinical-radiographic manifestations. Although almost half of patients follow a spontaneous resolution or under treatment, a significant number of them may have several degrees of organ damage. This study emphasizes the value of a multidisciplinary approach and long-term follow-up by specialized teams in sarcoidosis.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 28723801
OWN - NLM
STAT- MEDLINE
DA  - 20170720
DCOM- 20170808
LR  - 20170808
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 29
DP  - 2017 Jul
TI  - Multidisciplinary approach and long-term follow-up in a series of 640 consecutive
      patients with sarcoidosis: Cohort study of a 40-year clinical experience at a
      tertiary referral center in Barcelona, Spain.
PG  - e7595
LID - 10.1097/MD.0000000000007595 [doi]
AB  - Cohort studies of large series of patients with sarcoidosis over a long period of
      time are scarce. The aim of this study is to report a 40-year clinical experience
      of a large series of patients at Bellvitge University Hospital, a tertiary
      university hospital in Barcelona, Spain. Diagnosis of sarcoidosis required
      histological confirmation except in certain specific situations. All patients
      underwent a prospective study protocol. Clinical assessment and follow-up of
      patients were performed by a multidisciplinary team.From 1976 to 2015, 640
      patients were diagnosed with sarcoidosis, 438 of them (68.4%) were female (sex
      ratio F/M 2:1). The mean age at diagnosis was 43.3 +/- 13.8 years (range, 14-86
      years), and 613 patients (95.8%) were Caucasian. At diagnosis, 584 patients
      (91.2%) showed intrathoracic involvement at chest radiograph, and most of the
      patients had normal pulmonary function. Erythema nodosum (39.8%) and specific
      cutaneous lesions (20.8%) were the most frequent extrapulmonary manifestations,
      but there was a wide range of organ involvement. A total of 492 patients (76.8%) 
      had positive histology. Follow-up was carried out in 587 patients (91.7%), over a
      mean of 112.4 +/- 98.3 months (range, 6.4-475 months). Corticosteroid treatment
      was administered in 255 patients (43.4%), and steroid-sparing agents in 49
      patients (7.7%). Outcomes were as follows: 111 patients (18.9%) showed active
      disease at the time of closing this study, 250 (42.6%) presented spontaneous
      remission, 61 (10.4%) had remission under treatment, and 165 (28.1%) evolved to
      chronic sarcoidosis; among them, 115 (19.6%) with mild disease and 50 (8.5%) with
      moderate to severe organ damage. A multivariate analysis showed that at
      diagnosis, age more than 40 years, the presence of pulmonary involvement on chest
      radiograph, splenic involvement, and the need of treatment, was associated with
      chronic sarcoidosis, whereas Lofgren syndrome and mediastinal lymphadenopathy on 
      chest radiograph were indicators of good outcome.Sarcoidosis is a multisystem
      disease with protean clinical-radiographic manifestations. Although almost half
      of patients follow a spontaneous resolution or under treatment, a significant
      number of them may have several degrees of organ damage. This study emphasizes
      the value of a multidisciplinary approach and long-term follow-up by specialized 
      teams in sarcoidosis.
FAU - Mana, Juan
AU  - Mana J
AD  - aDepartment of Internal Medicine bDepartment of Dermatology cDepartment of
      Pulmonary dDepartment of Pathology eDepartment of Ophthalmology fDepartment of
      Neurology gDepartment of PET Unit-Institut de Diagnostic per la Imatge
      hUniversity of Barcelona iFaculty of Medicine and Health Sciences, Universitat
      Internacional de Catalunya jBellvitge University Hospital, Bellvitge Biomedical
      Research Institute-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain.
FAU - Rubio-Rivas, Manuel
AU  - Rubio-Rivas M
FAU - Villalba, Nadia
AU  - Villalba N
FAU - Marcoval, Joaquim
AU  - Marcoval J
FAU - Iriarte, Adriana
AU  - Iriarte A
FAU - Molina-Molina, Maria
AU  - Molina-Molina M
FAU - Llatjos, Roger
AU  - Llatjos R
FAU - Garcia, Olga
AU  - Garcia O
FAU - Martinez-Yelamos, Sergio
AU  - Martinez-Yelamos S
FAU - Vicens-Zygmunt, Vanessa
AU  - Vicens-Zygmunt V
FAU - Gamez, Cristina
AU  - Gamez C
FAU - Pujol, Ramon
AU  - Pujol R
FAU - Corbella, Xavier
AU  - Corbella X
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
SB  - AIM
SB  - IM
MH  - Academic Medical Centers
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - European Continental Ancestry Group
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multivariate Analysis
MH  - Patient Care Team
MH  - Prognosis
MH  - Prospective Studies
MH  - Respiratory Function Tests
MH  - Sarcoidosis/diagnostic imaging/epidemiology/pathology/*therapy
MH  - Spain
MH  - Tertiary Care Centers
MH  - Time Factors
MH  - Young Adult
PMC - PMC5521941
EDAT- 2017/07/21 06:00
MHDA- 2017/08/09 06:00
CRDT- 2017/07/21 06:00
AID - 10.1097/MD.0000000000007595 [doi]
AID - 00005792-201707210-00066 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Jul;96(29):e7595. doi: 10.1097/MD.0000000000007595.