Uncommon pneumoconioses

1. Uncommon pneumoconioses P. De Vuyst TTS 2007

2. Definition of Pneumoconiosis  « A reaction of the lung to inhaled inorganic dusts and the resultant structural changes which exclude neoplasia, asthma, bronchitis and emphysema » From a very limited reaction to a severe fibrosis causing death by respiratory failure Parkes, 1994

3. Will not be discussed Pleural disorders (although part of the ILO classification) Interstitial lung diseases due to organic dusts (extrinsic allergic alveolitis) and to "chemicals" (popcorn, polymers, nylon…) Relationship between IPF and inorganic dusts Systemic diseases associated with silica exposure (scleroderma, vasculitis, rheumatoid arthritis)

4. Epidemiology of pneumoconioses • Rare pneumoconioses • New potential risks for pneumoconiosis • How to differentiate pneumoconiosis from "idiopathic" lung disease ?

5. Incidence of pneumoconiosis Data from the Occupational Diseases Funds of Belgium (10.106 inhabitants) New cases accepted per year (2000-2006) Silicosis and CWP: around 100 cases/yr (1/100,000) Asbestosis (pleural plaques excluded): around 50 cases/yr (0.5/100,000)

6. Incidence of pneumoconiosis Silicate pneumoconiosis, cobalt and hard metals disease, berylliosis, aluminum lung, siderosis: all less than 1 case/yr (0.01/100,000) (2000-2004) Incidence in the same population of at least 1000 new cases of sarcoidosis and 500 of IPF… (Incidences extrapolated from the European Lung White Book, 2003)

7. The incidence of pneumoconiosis is underestimated Low-grade pneumoconiosis is asymptomatic and the progression of disease is usually very slow No medical surveillance of selfstanding or retired workers Insufficient knowledge of these diseases by clinicians Taking a « complete » occupational history needs time and expertise Underdeclaration of occupational diseases

8. Pneumoconioses in industrialized countries • The incidence of severe and symptomatic forms of pneumoconioses has decreased due to a reduction of workforce and to a substantial decrease in exposure levels (several orders of magnitude) • Most cases of advanced forms of pneumoconiosis result from exposures dating back several decades ago or unusual exposures

9. CWP in US 29,521 coal miners examined between 1996-2002 Incidence of CWP 3% (ILO 1/1) Progressive massive fibrosis: 0.15% Stage C: 0.01% (5 cases) Antao V, et al, ATS 2005

11. Man, 26 yrs-old (2003)

12. Occupational history • Works in his father industrial coachbuilder’s workshop since the age of 15 • Was sometimes present as a younger child • Sandblasting (quartz under pressure) of trucks and tanks • Plating (projection of heated Al and Zn ) • Episodes of metal fume fever • Used a protection (half-scaphander) …

14. January 2006

19. 200 x 1250 x

21. Epidemiology of pneumoconioses • Rare pneumoconioses • New potential risks for pneumoconiosis • How to differentiate pneumoconiosis from "idiopathic" lung disease ?

22. Minerals causing Pneumoconiosis  Asbestos fibers (asbestosis) Silica (silicosis) Coal mine dust (coal worker’s pneumoconiosis) Talc, kaolin, mica and other silicates: pure exposures are rare

23. Silicate pneumoconioses • Excessive dust accumulation (overload), is the main cause of disease • Incidence has decreased because of reduction of dust levels • Pure silicate exposures are rare and silica may be present (mixed dust fibrosis) • Asbestos (tremolite) may be present in talc or vermiculite

24. Talc pneumoconiosis • Talc is extracted from open mines and milled • Industrial uses: paints, paper, cosmetics, rubber, pharmaceutics… • Nodular (small rounded or large opacities) or diffuse interstitial pattern (irregular opacities)

27. Man, born in 1932 Talc miller from 1969 to 1980 (high grade talc for pharmaceutical uses) CXR in 1983 Abundant talc particles (and ferruginous bodies) in BAL Talc pneumoconiosis

29. Man, born 1954 Last expertise case from the same factory in 1994 Talc pneumoconiosis

30. Metals and alloys causing Pneumoconiosis  Cobalt and hard metals (WC), beryllium: rare but well-characterized entities Rare and less well-characterized entities: aluminum, "welder’s lung", "dental technician’s pneumoconiosis"… Rare and benign: iron, tin, barium

31. Beryllium and cobalt/hard metal pneumoconioses • Individual susceptibility plays a dominant role • No direct relationship with cumulative exposure • Diseases may occur after short periods • May improve after eviction and/or steroid treatment

32. Beryllium • Very light, solid and resistant metal used in high technology industries • Aerospace, ceramics, defense, electronics, prosthetic alloys, automotive and naval industries, sports… • 2-20% of exposed workers sensitize to beryllium (positive Be lymphocyte proliferation test)

33. Aluminum Beryllium Alloy Copper Beryllium Alloy

34. Beryllium alloy parts for electrical connectors in cell phones, computers, automobiles Beryllium-containing parts for high technology applications

35. Beryllium: In stereo speaker Beryllium-copper tambourine jingles Beryllium: Golf club

37. Chronic berylliosis Disease similar to sarcoidosis, occurring in less than 10% of exposed workers (Newman L & Maier L,Interstitial Lung Disease 2003, Schwarz & King, eds) Genetic susceptibility to immunologic sensitization: association between chronic berylliosis and HLA mutated genes (HLA DPB Glu 69 and DRB Phe 47) (Saltini C et al, Eur Respir J 1998)

39. Chronic Beryllium Disease • Latency between onset of exposure and disease : from weeks to several years ( up to 40 yrs) • Sensitization possible after 2 months and CBD after 3 months • Disease may occur in susceptible individuals after low dose or indirect exposures (bystanders, secretaries, neighborhood)

40. Chronic berylliosis • Non specific symptoms • Sometimes systemic disease (skin, liver, bone..) • Biological abnormalities: SACE, T4 lymphocytosis in BAL… • Pathology: epithelioid granulomas and lymphocytic pneumonia • Be is a too light element to be detected by classical techniques of mineral analysis

41. Detection of Be in lung tissue • LAMMA • SIMS Sawyer RT, Abraham JL, Daniloff E, Newman LS. Secondary ion mass spectroscopy demonstrates retention of beryllium in chronic beryllium disease granulomas. J Occup Environ Med 2005; 1218-1226.

42. Berylliosis : diagnosis • Exposure to Be under any form (metal, alloy, fumes) • Demonstration of an abnormal lymphocytic response (lymphocyte proliferation test) in vitro to Be salts • Presence of granulomas and/or lymphocytes in lung tissue • Treatment: eviction and steroids. Useful in symptomatic forms. Long-term impact ?

43. Hard metal and cobalt disease • Hard metals for cutting and drilling tools: tungsten carbide with 10-20% cobalt • Cobalt is the component that induces pulmonary disease, although it is rarely found in lung tissue because of its solubility • Same disease in diamond polishers using cobalt disks (Demedts M et al, Am Rev Respir Dis 1984)

44. Hypersensitivity pneumonitis and irreversible pulmonary fibrosis when exposure is continued Presence of bizarre multinucleated giant cells with "cannibalistic" cell-in-cell features in lung tissue and BALF are highly suggestive of cobalt disease (GIP) Hard metal and cobalt diseases E. Verbeken and B. Nemery, KU Leuven,

45. 29 year old man Worked with diamond-cobalt disks for 8 years as a diamond polisher Lung biopsy: GIP Died while awaiting lung transplantation Cobalt lung

47. Aluminium lung: rare disease • Bauxite smelting ( Shaver’s disease) • Workers exposed to aluminum powders in fireworks and explosives industry • Upper and mid zonal fibrosis, honeycombing, subpleural bullae (pneumothorax) • More cases detected by HRCT

48. « Aluminium lung » • 61 yr old metal polisher, principally aluminium, during 24 yrs. Very dusty work • Dyspnea, chest pain and cough • Restrictive defect with low DLCO (36 %) • Lung fibrosis and adenocarcinoma • No silicotic nodules • MA of lung tissue: aluminum and silica