sarcoidosis. a mysterious disease

Sarcoidosis.A Mysterious Disease.

Peter Martin.LADUCA.Wellington.26 March 2009.

Nearly always involves the lungs

Nearly always occurs in the lungs

Can involve almost any organ

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

At the end of this session you will:

Have an over view of possible causes

Understand the different types of lunginvolvement

Know of tests available to assess severity

Have an idea of other organ involvement

At the end of this session you will:

Have an over view of possible causes

Understand the different types of lunginvolvement

Know of tests available to assess severity

Have an idea of other organ involvement

Understand the significance for underwriters and claims assessors

The appearance microscopically is very characteristic but can also occurin 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

Used to be often confused with TB

Lung Involvement: Stage 1.

Erythema nodosum

Sometimes occurs withsarcoidosis especiallystage 1.

Can also occur in several otherdiseases.

Lung Involvement Stage 2.

Both lung and lymph nodeare diseased.

Can progress from stage 1or be stage 2 at presentation.

Less favourable prognosis.

Lung Involvement: Stage 3.

As with stage 2: lung changes – but no lymph node enlargement.

Less favourable prognosis than stage 2.

Lung Involvement: Stage 4

Extensive destruction andscarring.

Poor prognosis: often leadsto major disability or death.

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

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

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

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 erythemanodosum)

Heart:

Often damagesconducting pathways

Can cause suddendeath

Investigations:

EchocardiogramMRI

Sarcoidosis of Eye

Retina or optic nerve – as in picture

Anterior part of eye – painful red eye

Can cause blindness

What Causes Sarcoidosis?

What Causes Sarcoidosis?

I don’t know

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

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

Traps for Underwriters

Stage 1 disease has good chance of complete recovery

But can progress to stages 2 - 4

Seek evidence ofrecovery with examination bydoctor and X-ray

Traps for Underwriters

Stage 2 and especially stage 3 disease may lead to death ordisability

Usually require current assessment by a RespiratoryPhysician including lung function, biochemical tests,and X-ray or CT - loading

Usually need exclusions onIP or TPD and ? Trauma /Crisis

Traps for Underwriters

Extra-pulmonary disease

Most will need current assessmentby Respiratory Physician andother specialist eg. Cardiologist,Ophthalmologist

Most will need major exclusionseg. eye

Some, such as cardiac, may be un-insurable

Claims Assessors – Death Claims

Sudden death will usually bedue to cardiac disease

May be unheralded – clearlyinvestigate disclosures atapplication

For non-underwrittenpolicies – check earlierrecords

Claims Assessors – Death Claims

Usually due to advancedlung disease

Can occur from cardiacdisease or complications of treatment

Income Protection and TPD

Mostly arise from advancedlung disease

Usually check there hasbeen a recent assessmentby a RespiratoryPhysician – treatment including rehabilitation

Crisis / Trauma Claims

Organ transplantation usuallylung or heart

Blindness

Activities of daily living

Summary

Presents in many ways

Usually lungs

Any organ

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