SLICOSIS PROGRESSIVE MASSIVE FIBROSIS IGMC

PMF

1. SILICOSIS- PROGRESSIVE MASSIVE FIBROSIS –A Diagnostic Challenge D r Aabha Singh,Dr Madan Lal Kaushik Junio r R esident Departmen t of Medici n e Proffessor Department of Medicine INTRODUCTION SILICOSIS is a fibrosing disease of lung caused by inhalation retention and pulmonary reaction to silica . Occupational exposure to silica particles of respirable size 0.5-5um has classically been associated with mining, quarrying, tunneling, abrasive blasting, construction, and foundry work .Even brief periods of exposure to high levels of freshly fractured silica can result in a lifetime increased risk for disease. CASE PRESENTATION 43 year old male working in a tunnel for last 11.5 years presented with C/C of SOB on walking slight uphill for last five months which was increased for last five days s/t patient was dyspneic even at rest. Fever and blood streaked sputum for last five days INVESTIGATIONS ACE 119 Sputum for cbnaat neg BAL negative for TB PFT restrictive pattern ANA by hep 2 Anti GBM ANTI PR3 - Neg Lung biopsy - histopath report s/o ILD Discussion Crystalline silica interacts with aqueous media leading to generation of free radicals and also directly inju r ed pulm o na r y mac r opha g es leadin g t o ch r o n ic lung inflammation and fibrosis , Can present as Ac u t e : Acce l e r a t ed : Ch r o n ic silic osis Acut e sili cosis – CXR - bilateral diffuse opacities peri hilar or basilar region HRCT - centrilobular nodular opacities crazy pa v eme n t p a tter n can als o b e seen Accele r a t ed / ch ron i c sili cosis Cxr inumerable small opacities in bilateral upper z o n es of lung H R C T bil a t e r a l s y m metric centrilobula r perilymph a ti c n o dules Eg g shell calcific a tio n of lymp h n o des PM F - conglome r a t es lesions an d emph y sem a t ous chan g es can b e seen Raised ACE levels - increased degradation of pulmonary macrophages which is a common source of ACE in sarcoidosis and silicosis . Higher levels are associated with disease severity of silicosis its progression to PMF and its radiological progression. Silicotuberculosis The relative risk to develop tuberculosis - 2.8 X and extra pulmonary TB 3.7 X higher Silica is a group I carcinogen increases the risk of lung cancer Silicosis increases the risk of CTD most commonly associated are Scleroderma , RA , SLE. BIBLIOGRAPHY Elkard I, et al La silicotuberculose. Revue De Pneumologie Clinique. 2016 . Szechiński J,et al. Serum angiotensin ‐ converting enzyme levels in patients with silicosis. Journal of Toxicology and Environmental Health, Past history - Patient presented in pulmonary department 5 months back for similar complaints for which bronchoscopy was done which was negative for TB CT - s/o bilateral upper lobe fibrosis On examination spO2 56% at room air RR 30/min Cyanosis present Auscultation- Bilateral air entry decreased With end inspiratory crepts in bilateral infraclavicular , mammary and infrascapular area. CXR HRCT CHEST