Anne Llewellyn examines the consequences and underwriting ramifications of this little known disease.
Sarcoidosis is a progressive, inflammatory disease of unknown cause characterised by the formation of granulomas, abnormal nodules of inflamed tissue that can affect any organ, altering its structure and function.
It primarily affects young and middle aged adults. Around 20% of those diagnosed with sarcoidosis will experience functional impairment, and the mortality rate is about 5%. A disclosure of sarcoidosis is therefore significant at underwriting stage, but as 80% of sufferers have no impairment the majority of applicants with this condition will be accepted at standard rates or with a small loading.
Our job is to identify the small percentage that present an unacceptable risk for life and health insurance, and ensure the best cases are given appropriate terms.
What is Sarcoidosis?
The cause of sarcoidosis is unclear. Although it is sometimes referred to as an autoimmune condition, to date no convincing evidence links a specific self antigen to sarcoidosis. It is possible that sarcoidosis may be caused by different mechanisms, such as environmental agents (fungus, bacteria or virus), certain infections, and genetic predisposition.
Medical opinion remains divided and there have been no studies which have been successful in isolating a single cause, which might explain why the disease is so different from one individual to the next.
Sarcoidosis usually initially affects the lungs and lymph nodes in the chest cavity. White blood cells accumulate at the site of inflammation, forming nodules called granulomas which clump together. As the granulomas grow they start to affect the function of organ in which they are accumulating, and the patient will experience symptoms. About 5% of sarcoidosis cases are diagnosed on routine chest X-ray before symptoms are even evident.
The course of the disease varies greatly from patient to patient; the granulomas may continue to grow, they may grow very slowly or they may stop, or even regress. In come cases permanent scarring is left which continues to impair organ function even after the disease is no longer active.
Although the majority of cases start in the lungs, sarcoidosis usually spreads to one or more other organs, most commonly the eyes and skin, although the liver, spleen, brain, heart, peripheral nerves, kidneys, tear and salivary glands and bone and joint tissues can also be affected.
Symptoms
Symptoms depend on the extent and severity of the organ involved. They usually appear gradually, and range from very mild to a debilitating chronic condition.
About half of sarcoid patients will first present with shortness of breath, wheezing, chest pain, and a persistent cough. Systemic complaints of fever, lack of appetite and joint pain occur in just under half of cases.
Although granulomas usually occur in other organs, they are often symptomless, but depending on the organ involved, further symptoms could include:
• Eyes - dry eyes, blurry vision, pain, sensitivity
• Skin - dermatological granulomas occur in about 25% of cases - may be painful and may leave scars
• Nerves - facial nerve palsy, tingling, headache, symptoms mimicking a tumour
• Heart -syncope (loss of consciousness) or difficulty breathing due to heart failure
• Liver - jaundice
• Kidneys - pain and stone formation
Diagnosis of sarcoidosis isn't always easy, as many other conditions cause similar symptoms, so diagnosis is usually one of exclusion of alternative diagnoses. Granulomas in the lungs and around the lymph nodes in the chest can be seen on chest x-ray. Examining a sample of tissue taken from affected skin or lung under the microscope usually confirms the diagnosis.
Treatment
The exact percentages vary according to different studies, but 60 - 80% of cases go into remission without being treated. For the rest, as the cause is unknown it is difficult to treat and there is no cure. Treatment is therefore prescribed to alleviate the symptoms.
Systemic corticosteroids are the mainstay of sarcoidosis treatment. Corticosteroids impede the formation of granulomas and are therefore largely efficient against most active clinical manifestations. However, there are substantial side effects associated with systemic corticosteroids, and relapses occur when withdrawal is too rapid.
Some patients cannot tolerate corticosteroids and others do not respond to them. In addition while corticosteroids work for most people in the short term, alleviating symptoms caused by inflammation and swelling, there is no evidence of the long term benefit. They do not, for example, prevent the formation of lung fibrosis.
Non-steroidal anti-inflammatory drugs such as ibuprofen can be effective in treating mild symptoms, and there are stronger alternatives to corticosteroids such as methotrexate and azathioprine which suppress the immune response, cyclophosphamide (usually used to treat cancer) and chloroquinine phosphate, an anti malarial used to treat skin manifestations of sarcoidosis. These drugs each have side effects some of which are undesirable and methotrexate, azathioprine and cyclophosphamide are toxic and therefore not given unless necessary.
Prognosis
The prognosis for the majority of sarcoid suffers is extremely good. Approximately half of the cases resolve or can be cured within 12-36 months and most within five years. In many cases the disease can remit spontaneously without the need for treatment. However, in others it becomes chronic and may persist for several decades.
Pulmonary fibrosis is the most frequent severe manifestation, accounting for the major cause of morbidity and mortality in western countries.
Complications, although rare, include:
Eyes - glaucoma, cataracts, loss of visual acuity, blindness
Neurological - visual and hearing difficulties, meningitis
Cardiac - heart failure, heart block, sudden death
Renal - kidney failure
Underwriting Implications
There are four stages of sarcoidosis, not necessarily progressive, which are categorised by chest x-ray changes:
Stage 0: Normal chest x ray
Stage 1: Chest x ray showing enlarged lymph nodes but otherwise clear lungs
Stage 2: Chest x ray showing enlarged lymph nodes and mild infiltrates in the lungs
Stage 3: Chest x ray showing infiltrates but the lymph nodes are not enlarged
Stage 4: Chest x ray showing evidence of pulmonary fibrosis in the lung tissue.
An underwriting decision will usually be made on a combination of factors with the staging as the basis for the rating. We will also take into account the time since diagnosis, the history of the disease, whether currently active or in remission, treatment, organ involvement, and the results of clinical tests such as pulmonary function tests and ECGs where available.
If the diagnosis is recent, usually within the last six months, an application will be postponed. After that period, a stage one or mild disease which is active and currently on treatment will usually attract a rating of no more than +50%-100% for life and critical illness cover. For stage 2 or moderate disease ratings will be slightly higher. Cases in remission or with no progression over time can usually be accepted at standard rates. Ratings will increase depending on the rate of progression and organ involvement. The most severe cases will be declined.
Terms can even be offered for income protection on the best cases, particularly where lesions are regressing and the condition was only mild.
Anne Llewellyn is underwriting development manager at PruProtect
SOURCES
Kamangar, N., 2009. Sarcoidosis http://emedicine.medscape.com/article/301914-overview [accessed 5 November 2009]
Nunes, H. et al.,2007. Sarcoidosis http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2169207/?tool=pmcentrez [accessed 5 November 2009]
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http://emedicine.medscape.com
Underwriting implications - RGA, Pacific Life Re
