ArticleOrphanet journal of rare diseases2018
Comorbid connective tissue diseases and autoantibodies in lymphangioleiomyomatosis: a retrospective cohort study.
Article in Orphanet journal of rare diseases, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed, 7 citations in OpenAlex.
- Lymphangioleiomyomatosis with superimposed organizing pneumonia diagnosed by transbronchial lung cryobiopsy: A case report.Respiratory medicine case reports · 2026Article
- An Unusual Case of Primary Sjögren's Syndrome and Lymphangioleiomyomatosis Diagnosed From Refractory Cough.Cureus · 2025Article
- Rab4A-directed endosome traffic shapes pro-inflammatory mitochondrial metabolism in T cells via mitophagy, CD98 expression, and kynurenine-sensitive mTOR activation.Nature communications · 2024Article
- An Evidence-Based Update on the Potential Association between Rheumatoid Arthritis and Lymphangioleiomyomatosis.Journal of personalized medicine · 2023Review
- Cell type-specific mechanistic target of rapamycin-dependent distortion of autophagy pathways in lupus nephritis.Translational research : the journal of laboratory and clinical medicine · 2022Review
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundLymphangioleiomyomatosis (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.
methodsA 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.
resultsAll 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.
conclusionsComorbid CTDs, especially SjS, in LAM patients should be considered.
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