While half of all sufferers make a full recovery, the remainder can have permanent lung problems. Vanessa Leyland lists the tell-tale signs of sarcoidosis
Sarcoidosis in an inflammatory disorder that is characterised by small rounded growths made up of blood vessels, cells and connective tissue - also known as granulomas or granulomata.
Although the cause of the disease remains a mystery, it is thought that the inflammation is caused by a reaction to a threat - for example, a viral infection, a fault with the immune system, an anonymous noxious substance or environmental factor, or even an inherited or genetic cause.
Sarcoidosis can affect any organ and it will always show in more than one. Organs most likely to be infected include the liver, skin, eyes and most commonly the lung and lymph nodes, which may cause chest pain.
Symptoms
Symptoms include fever, tiredness and an overall feeling of being unwell. Some people only have slight symptoms, or even none at all - this really depends on the organs affected:
n Liver: bile collecting in the liver, or enlargement of the liver occurs in 25% of cases.
• Skin: blotchy red skin or thickened purple lesions on skin occur in 25% of cases. Sarcoidosis is the most common cause of erythema nodosum - small red nodules that can be extremely sore - which appear on the shins or lower limbs, slowly fading to leave a bruised appearance.
• Nervous System: partial facial paralysis, loss of hearing, fits - sarcoidosis can involve any part of the nervous system, though involvement of the central nervous system is rare.
• Eyes: visual defects, such as conjunctivitis, arise in 20% of cases - this may develop into blindness. Calcium deposits in the cornea can occur if there is too much calcium present in the blood.
• Heart: occasionally this may not be noticed, although frequently there are ECG irregularities and heart enlargement that can possibly lead to heart failure.
• Joints: arthritis and inflammation of the joints occurs in 50% of patients.
• Lymph nodes: sarcoidosis mainly occurs in the lymph nodes in the lungs and in the lungs themselves.
• Kidney: due to raised calcium in the blood, kidney stones and calcium deposits occur.
• Endocrine: 10% of patients may have too much calcium in their blood, known as hypercalaemia, and most experience hypercalcuria, which is too much calcium in the urine. This is due to high levels of vitamin D.
Doctors will look for enlarged lymph nodes, a dry cough, skin redness and eye problems. Following that, a chest x-ray and tissue biopsy is taken from the lungs, lymph nodes or skin lesions.
Treatment
Treatment involves physiotherapy and anti-inflammatory drugs. Sometimes steroids are required. These can have an adverse effect on some people, resulting in increased appetite, weight gain and bruising. They are, therefore, given at the lowest dose to be effective for the shortest period possible.
Steroids can also have some psychological effects and can cause high blood pressure, diabetes or softening of the bones - also known as osteoporosis - and cataracts in the eyes. And, very rarely, a hip replacement may be required due to aseptic necrosis of the hips, which is the development of degenerative changes in the hips.
For people who are unable to tolerate the steroid dosage, they may be able to take another medicine called Methotrexate, then after six months they can decrease the steroid use. In some patients, however, Methotrexate can produce abnormal blood test results and some infections.
When treatment is not effective and the disease becomes life threatening, the worst-case scenario may be lung transplantation. However, most patients do not require steroids and sarcoidosis will eventually cease - even when the organs may have been damaged, the damage will be mild.
The outlook is not as good if there has been:
• Weight loss
• Dyspnoea (uncomfortable and increased breathing)
• A reduction in lung function
• Onset later than age 40
n Symptoms lasting over six months
n Involvement of more than three organs and lung disease
• In 15% of cases, it remains active or remitting/relapsing
Classifications
The condition has several different disease classifications. These include Lofgrens Syndrome, where the sarcoidosis presents itself as erythema nodosum; that is, with small red nodules present on the lower limbs, aching joints and swollen hilar lymph glands.
Another form is Heerfordt-Waldenstrom Syndrome, which consists of a salivary gland inflammation as well as an inflammation of the middle coat of the eye - which is known as uveitis - as well as facial nerve paralysis.
A third type of sarcoidosis is pulmonary - which can be progressive and result in dyspnoea and eventually heart disease, caused by disease in the lungs, and ultimately death.
A chest x-ray would show a mottling in the lungs, similar to a honeycomb, which is the fibrosis. The severity is then recorded in three stages, which have been set out by the International Congress on Sarcoid:
• Stage 1 - normal chest x-ray and active alveolitis, inflammation of the alveoli in the lungs
• Stage 2 - enlarged lymph nodes in the hilar regions of the lungs, known as hilar lymphadenopathy
• Stage 2A - hilar lymphadenopathy with disease infiltrating in the lungs
• Stage 2B - lung disease without lymph node enlargement
• Stage 3 - fibrosis in the lungs without disease in the lymph glands of the lungs
Up to 10% of people suffering with sarcoidosis die, which is usually due to cardiac involvement.
Vanessa Leyland is a life and disability underwriter
