Background Pulmonary involvement is still regarded as a common cause of morbidity in Polymyositis/Dermatomy
ositis. Interstitial lung disease can result in potentially fatal consequences such as ventilatory failure, secondary pulmo
nary arterial hypertension, or cor pulmonale. Early diagnosis of interstitial lung disease is hence a top priority in Poly
myositis/Dermatomyositis patients. Krebs von den Lungen-6 is a transmembrane mucoprotein that has recently
been identified as a promising marker for interstitial lung disease diagnosis and progression. As a result, it is regarded
as a powerful predictor of interstitial lung disease severity. Thirty polymyositis/dermatomyocitis patients were enrolled
in this study. Thirty age and sex matched healthy individuals were selected as control group. Cutaneous Dermatomy
ositis Disease Area Severity index(CDASI) was used for evaluation of skin severity, KL6 was measured using Elisa kit,
High-resolution computed tomography, pulmonary function tests were made.
Results Sixteen female and 14 male patients had a mean age of 41.64–8.02 and amedian of the disease
duration of 4 years. Fifteen patients (50%) had normal readings on High-resolution computed tomography
of the chest HRCT chest, while the other half had significant HRCT chest findings. KL-6 concentrations were seen
to be higher in Polymyositis/Dermatomyositis patients mainly those with ILD (mean ± SD 38.66 ± 22.98), compared
to the control group (Mean ± S 589.04 ± 409) and in patients without ILD (Mean ± SD 86.70 ± 8.99), (p < 0.001).
KL-6 serum concentrations were shown to have a significant connection with the HRCT score (r = 0.803, P < 0.001).
Forced vital capacity (FVC%) (r = − 0.910, P < 0.001), forced expiratory volume in 1 s (FEV1%) (r = − 0.767, P < 0.001),
and FEV1/FVC% (r = − 0.228, P = 0.112) were all inversely related to KL-6 concentrations.
Conclusion Individuals with PM and DM with concomitant ILD have increased KL-6 serum concentrations
in comparison to myositis individuals without ILD, denoting its potential role in diagnosis and follow up of PM/DM
with ILD. Further studies are needed to discuss the role of KL6 in large sample of the patient’s population, and its
correlation with other organ affection rather than ILD. Also to clarify the potentiality of adding the KL6 biomarker
to the guidelines of treat to target for Rheumatic diseases with lung affection and to see the effect of treatment
on KL6 serum level. |
