sarcoidosis. a mysterious disease
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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 - PowerPoint PPT PresentationTRANSCRIPT
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