Comorbid connective tissue diseases and autoantibodies in lymphangioleiomyomatosis: a retrospective cohort study

Shinji Futami,
Toru Arai,
Masaki Hirose,
Chikatoshi Sugimoto,
Naoya Ikegami,
Masanori Akira,
Takahiko Kasai,
Masanori Kitaichi,
Seiji Hayashi and
Yoshikazu InoueEmail author View ORCID ID profile

Orphanet Journal of Rare Diseases201813:182

https://doi.org/10.1186/s13023-018-0933-0

Received: 28 August 2018
Accepted: 9 October 2018
Published: 20 October 2018

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 1:40 or higher, and those of 1:160 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.