Sarcoidosis. A Mysterious Disease.

1. Sarcoidosis.A Mysterious Disease. Peter Martin. LADUCA. Wellington. 26 March 2009.

2. Nearly always involves the lungs

3. Nearly always occurs in the lungs Can involve almost any organ

4. Nearly always occurs in the lungs Can involve almost any organ Varies in severity from a trivial, short-lived illness to a severe and life threatening or disabling condition

5. At the end of this session you will: Have an over view of possible causes Understand the different types of lung involvement Know of tests available to assess severity Have an idea of other organ involvement

6. At the end of this session you will: Have an over view of possible causes Understand the different types of lung involvement Know of tests available to assess severity Have an idea of other organ involvement Understand the significance for underwriters and claims assessors

7. The appearance microscopically is very characteristic but can also occur in many other diseases; infections eg. TB at the periphery of a tumour Hence diagnosis usually depends on weighing up the results of many investigations – X-rays, biopsy, lung function tests, blood tests

8. Used to be often confused with TB

9. Lung Involvement: Stage 1.

10. Erythema nodosum Sometimes occurs with sarcoidosis especially stage 1. Can also occur in several other diseases.

11. Lung Involvement Stage 2. Both lung and lymph node are diseased. Can progress from stage 1 or be stage 2 at presentation. Less favourable prognosis.

12. Lung Involvement: Stage 3. As with stage 2: lung changes – but no lymph node enlargement. Less favourable prognosis than stage 2.

13. Lung Involvement: Stage 4 Extensive destruction and scarring. Poor prognosis: often leads to major disability or death.

14. Can progress through stages 1 – 4 or present at any stage.

18. Lung Function Tests. • Almost always required. • Spirometry – can have obstruction similar to asthma or COPD • Volumes – restrictive pattern in Stages 2 – 4 • Diffusing capacity – usually reduced in stages 2 – 4 • Lung function is most useful serial measurement

19. Biochemical Markers. • Liver function is often abnormal – reflects sarcoid involvement of liver • Serum calcium – sometimes elevated – can lead to kidney stones, renal failure, or cardiac arrythmias (avoid calcium or vitamin D supplements) • Angiotensin converting enzyme (SACE) – very useful index of disease activity

20. Remember: Lung involvement is most common Any organ can have deposits of sarcoidosis After lung, commonest are lymph nodes, liver and skin (in addition to erythema nodosum)

21. Heart: Often damages conducting pathways Can cause sudden death Investigations: Echocardiogram MRI

23. Sarcoidosis of Eye • Retina or optic nerve – as in picture • Anterior part of eye – painful red eye • Can cause blindness

25. What Causes Sarcoidosis?

26. What Causes Sarcoidosis? I don’t know

27. Treatment • Only well evidenced treatment is cortico-steroids – usually prednisone • Not usually indicated in stage 1 • Unlikely to be effective in stage 4 • Often required in stages 2 and 3 • Not always effective - usually stopped if no improvement • May be required for many years – hence complications of treatment • Steroids nearly always needed for extra-pulmonary disease

28. Less Well Founded Treatments • Inhaled steroids – sometimes help if there is airflow obstruction • Immune suppressive agents such as methotrexate or cyclosporin – usually a treatment of last resort • Eye disease can be treated with topical steroids

29. Traps for Underwriters Stage 1 disease has good chance of complete recovery But can progress to stages 2 - 4 Seek evidence of recovery with examination by doctor and X-ray

30. Traps for Underwriters Stage 2 and especially stage 3 disease may lead to death or disability Usually require current assessment by a Respiratory Physician including lung function, biochemical tests, and X-ray or CT - loading Usually need exclusions on IP or TPD and ? Trauma /Crisis

31. Traps for Underwriters Extra-pulmonary disease Most will need current assessment by Respiratory Physician and other specialist eg. Cardiologist, Ophthalmologist Most will need major exclusions eg. eye Some, such as cardiac, may be un-insurable

32. Claims Assessors – Death Claims Sudden death will usually be due to cardiac disease May be unheralded – clearly investigate disclosures at application For non-underwritten policies – check earlier records

33. Claims Assessors – Death Claims Usually due to advanced lung disease Can occur from cardiac disease or complications of treatment

34. Income Protection and TPD Mostly arise from advanced lung disease Usually check there has been a recent assessment by a Respiratory Physician – treatment including rehabilitation

35. Crisis / Trauma Claims Organ transplantation usually lung or heart Blindness Activities of daily living

36. Summary • Presents in many ways • Usually lungs • Any organ

37. Summary • Lung disease shows spectrum from trivial, self-limiting to severe disabling and life-threatening • More severe disease shows variable response to treatment • Underwriters beware extra-pulmonary disease • Claims assessors should check for evidence of earlier presentations