Monir Hossain1, AHM Raihanuzzaman Sarkar1, ASM Fateh Akram1, Bashir Ahmed2,Md. Khairul Hassan Jessy2

Abstract
Scleroderma-related interstitial lung disease (SSc-ILD) is a fibrosing disorder of lung characterized by
systemic inflammation and progressive scarring of the lungs that leads to respiratory failure. There is no
definitive treatment for SSc-ILD though certain immunosuppressive therapies may slow disease progression.
Despite recent advancement of treatment it is the major cause of morbidity and mortality. An early diagnosis
of ILD is possible and is mandatory to improve the prognosis of the disease. Here we report a case of 36
years old male who presented with cough, painful joint swelling and breathlessness. HRCT of chest showed
reticulonodular opacity and ground glass haziness more marked at bases of lung with traction bronchiectasis.
Spirometry with DLCO showed severe restrictive ventilatory and diffusion defect. ANA and Anti Scl-70 was
positive.Understanding the mechanism of disease initiation and progression,effective theraputic options
are still limited.
Keywords: Pulmonary fibrosis, Progressive systemic sclerosis, Immunosuppression.
Abbreviations: HRCT: High resolution computed tomography, ILD: Interstitial lung disease, IIP: Idiopathic
interstitial pneumonia, IPF- Idiopathic pulmonary fibrosis, ANA- Anti nuclear antibody, SSc-Systemic
sclerosis, IP: Interphalangial, MCP: Metacarpophalangial, MTP: Metatarsophalangial, AFB:Acid fast bacilli,
MT: Manteux test, PASP: Pulmonary arterial systolic pressure, MWT: Minute walk test, ABG: Arterial
blood gas.

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