CHEST RADIOLOGY

HRCT Lung images of a 52 year old woman presenting with dyspnoea are shown below.What is the diagnosis?

Diagnosis

Scleroderma

The images shown reveal subpleural and basal predominant interlobular septal thickening, ground glass opacities, interstitial fibrosis with honeycombing. These features are seen also with Idiopathic pulmonary fibrosis or usual interstitial pneumonia (UIP).
There is dilatation of lower osephagus which clinches the diagnosis of scleroderma.

Scleroderma (also called “systemic sclerosis”) is a chronic autoimmune disorder. It is a poorly understood illness that causes widespread hardening of the skin, especially on the hands and face.  It may also damage the lungs, heart, kidneys, digestive tract, muscles, and joints

Types of Pulmonary Involvement
About 10% to 15% of people with scleroderma develop severe lung disease during the course of their illness.  

Two main clinical manifestations of lung involvement in scleroderma are:
1. Interstitial lung disease (also called fibrosing alveolitis or pulmonary fibrosis) 
2. Pulmonary vascular disease, leading to pulmonary hypertension. 

Fibrosing alveolitis occurs in more than three-quarters of patients with scleroderma, and vascular disease in at least 10 percent and possibly many more. 
The incidence of pulmonary hypertension has varied from 10 to 80 percent.  It occurs most frequently as an isolated pulmonary complication, but can be secondary to pulmonary fibrosis. 
Pulmonary hypertension is typically subclinical for many years until the on-going arterial damage results in the new onset of dyspnea.
There are also a number of less common manifestations of pulmonary involvement in scleroderma.  This may include pleural disease, aspiration pneumonia, bronchiectasis, spontaneous pneumothorax, drug-associated pneumonitis, and neoplasm.

Dr Paresh Desai, Goa