Comorbid connective tissue diseases and autoantibodies in lymphangioleiomyomatosis: a retrospective cohort study.
Orphanet J Rare Dis
; 13(1): 182, 2018 10 20.
Article
em En
| MEDLINE
| ID: mdl-30342527
ABSTRACT
BACKGROUND:
Lymphangioleiomyomatosis (LAM) and connective tissue diseases (CTDs) occur more frequently among women than men. We investigated the frequency of comorbid CTD and positive serum autoantibody findings in patients with LAM.METHODS:
A total of 152 patients with LAM were prospectively and consecutively registered in the National Hospital Organization Kinki-Chuo Chest Medical Centre cohort. The clinical data were retrospectively analysed, and patients were categorised into the following three groups a CTD group, a non-CTD-autoantibody-positive group, and a non-CTD-autoantibody-negative group.RESULTS:
All patients were women. We identified five patients with comorbid CTDs (3.3%) Sjögren's syndrome (SjS) (n = 3), systemic lupus erythematosus (n = 1), and rheumatoid arthritis (n = 1). One patient with SjS was also diagnosed with antiphospholipid antibody syndrome. The positive rate for anti nuclear antibody was 31.5% and 6.9% at dilution of 140 or higher, and those of 1160 or higher, respectively. It tended to be lower in patients with LAM than in healthy women. The positive rate for anti-SS-A and anti-SS-B antibody was 7.9% and 1.8%, respectively. No significant differences in age, type of LAM, smoking status, serum vascular endothelial growth factor D level, respiratory function, treatment, or prognosis were observed among the three groups.CONCLUSIONS:
Comorbid CTDs, especially SjS, in LAM patients should be considered.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Autoanticorpos
/
Comorbidade
/
Linfangioleiomiomatose
/
Doenças do Tecido Conjuntivo
Tipo de estudo:
Etiology_studies
/
Incidence_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Adult
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Female
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Humans
/
Middle aged
Idioma:
En
Revista:
Orphanet J Rare Dis
Assunto da revista:
MEDICINA
Ano de publicação:
2018
Tipo de documento:
Article
País de afiliação:
Japão