Introduction
There are more than 100 dissimilar autoimmune and inflammatory
circumstances in children distressing the muscles, cartilage, joints,
bones, and skin 1,2. 294,000 children
in the US have been diagnosed with a rheumatologic condition as assessed
by Centers for Disease Control (CDC) which regularly first appears
between the ages of 5 and 10 years, and girls are affected twofold as
often as boys 3, yet prevalence in
developing country not correctly estimated. Any organ system can be ed
by CTD with a broad variety of disease features and severity, such as
rash, arthritis, and more advanced presentation as renal or respiratory
failure. The identification of CTD is frequently delayed and identifying
the exact nature of CTD can be challenging4 . In SLE, about half of patients have
some form of pulmonary immersion throughout the progression of the
disease 5-8.
Interstitial lung diseases (ILD) are a cluster of disorders
characterized by interstitial inflammation and fibrosis, The prevelance
of chILD was approximated annually; 1.3 to 3.6 per 1,000,000 in children
under 17 years, 108–162 per 1,000,000 in children under 15 years9-11. The large disparity noted can be clarified by
the difference in the population involved in each study and a difference
in the criteria of ILD diagnosis. CTD related ILD (CTD-ILD) occurs with
development of original disease with difference prevelance12. ILD is the typical presentation of
respiratory system involvement in autoimmune connective tissue disease,
it’s not limited to the interstitium but also alveoli, pleura, vascular,
lymphatic structures, and airways of all diameters may all be included
in the disease. ILD when present, is frequently accompanying by
significant morbidity and mortality 6.Similarly, 8,13 mentioned that
Interstitial lung disease (ILD) is one of the crucial issues that impact
the prognosis of connective tissue diseased patients, and can cause
uninhibited systemic disease activity in CTD, it can be fatal, and early
detecting of the disease is, subsequently, important. Pulmonary function
tests are key in assessing CTD patients, PFT inform disease severity at
first diagnosis and response to therapy over follow up14 . High-resolution computed
tomography (HRCT) is gold corner to diagnose ILD12,15. The pathogenesis of ILD in
rheumatic disease is complicated, multifactorial, and incompletely known16-18. The clinical manifestation and
progression of ILD involves both inflammatory/immune and fibrotic/tissue
components, which often represent a progressive uncontrolled tissue
repair reaction in response to injury, finally leading to irreversible
remodelling of the lung and diminished lung function