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

Table 1 Characteristics of the 152 patients with LAM

	All	CTD	Autoantibody positive *	Autoantibody negative *	p-value †
Number of patients	152	5	33	114	–
Sex
Male / Female	0 / 152	0 / 5	0 / 33	0 / 114	1.00 ‡
Age at diagnosis, years §	40 (34–47)	48 (44–49)	42 (35–46)	38 (33–47)	0.22 \|\|
Type of LAM
Sporadic /TSC	131 / 21	5 / 0	31 / 2	95 / 19	0.10 ‡
Smoking history
Current / Ex / Never	6 / 29 / 117	0 / 0 / 5	0 / 6 / 27	6 / 23 / 85	0.40 ‡
Serum VEGF-D, pg/ml §	1652 (729–3183)	1413 (1048–1912)	1641 (615–3028)	1664 (762–3433)	0.53 \|\|
Respiratory function, %
%FVC §	99.4 (85.3–113.0)	91.6 (87.2–95.1)	99.7 (76.4–118.0)	100.0 (85.4–111.6)	0.66 ¶
%FEV₁ §	79.4 (61.5–105.7)	77.3 (72.8–86.0)	80.3 (56.5–107.8)	79.4 (63.0–104.2)	0.89¶
%DLco §	60.6 (39.6–82.5)	59.1 (35.1–71.2)	55.1 (38.2–74.0)	62.2 (41.9–84.7)	0.31 \|\|
Treatment
mTOR inhibitor + anti-oestrogen therapy	10	0	4	6	0.51 ‡
mTOR inhibitor only	45	1	10	34
Anti-oestrogen therapy only **	10	0	1	9
No treatment	87	4	18	65
Prognosis
Transplantation / dead	9 / 9	1 / 0	1 / 3	7 / 6	1.00 ‡
Alive without transplantation	135	4	30	101

* Autoantibody: anti-nuclear antibody 1:160 or higher, rheumatoid factor, anti-Ro antibody, anti-La antibody, anti-neutrophil cytoplasmic antibody, anti-double stranded DNA antibody, anti-topoisomerase antibody, anti-centromere antibody, anti-U1-ribonucleoprotein antibody, anti-Smith antibody, anti-cyclic citrullinated peptide antibody, anti-aminoacyl-tRNA synthetase antibody, and anti-histidyl-tRNA synthetase antibody
^† p-value is calculated between CTD plus autoantibody-positive and autoantibody-negative groups
^‡ Fisher’s exact test, ^§ Median (interquartile range), ^|| Mann-Whitney U-test, ¶ Student’s t-test
** Anti-oestrogen therapy: gonadotropin-releasing hormone (GnRH) analogue (n = 15), and/or anti-oestrogen drug (n = 3), and/or pro-gestational drug (n = 4), or ovariectomy (n = 1), or radiation therapy to ovary (n = 1)
Abbreviations: LAM lymphangioleiomyomatosis, CTD connective tissue diseases, TSC tuberous sclerosis complex, VEGF-D vascular endothelial growth factor-D, %FVC percent predicted forced vital capacity, %FEV₁ percent predicted forced expiratory volume in 1 s, %DLco percent predicted diffusing capacity of the lung carbon monoxide, mTOR mammalian target of rapamycin

ISSN: 1750-1172