THE FUNDAMENTALS OF SARCOIDOSIS

 What is Sarcoidosis?

 What causes Sarcoidosis?

 What is the course of the disease?

 What are the symptoms of Sarcoidosis?

 How is Sarcoidosis diagnosed?

 How is Sarcoidosis treated?
 

What is Sarcoidosis?

Sarcoidosis is a rare disease (in the region of 30 per 100,000 in the USA) that may affect one or more organs.^{1,2}  In a high proportion of cases it affects the lungs, eyes, skin, and lymph glands.^{3}  Either one or several of these may be affected.  The name for the disease is derived from the abnormal lumps or nodules (called granulomas) which form in one or more organs of the body.  They are sometimes referred to as “non-caseating granulomas.”  “Caseating” literally means milky, and the approximate meaning of the qualifying adjective is “non-softening.”^{2}  The granulomas may change the function of the affected organ.  Calcified spots on the lungs can be seen on computer tomography scans, but similar ‘shadowing’ is caused by other illnesses, such as TB, so a definitive diagnosis requires microscopic examination of affected tissue.^{3}

What causes sarcoidosis?

The cause of sarcoidosis is not known.  In recent years, an unusual type of bacteria, Cell Wall Deficient bacteria, has emerged as a possible candidate, but they are hard to see and do not easily succumb to antibiotics.^{6}  The predominant understanding has been that it is an autoimmune disease, namely one in which the body’s normal immune response goes haywire, so it starts damaging the body’s own tissue.  In sarcoidosis, the immune cells, which would normally be fighting an ‘invader’, clump together to form the aforesaid granulomas (small lumps or nodules).^{1,2,4}

What is the course of the disease?

The disease seems to follow two patterns, either acute or chronic.  For about 80% of people it is short lived, lasting about a couple of years, after which it disappears, never to return; and for another 10% it does not become serious.^{3}  This acute group tend to get a rapid onset of symptoms.  For the remaining 10%, whose symptoms generally appear gradually, it becomes chronic, and problems tend to get worse unless they can be halted by drugs.  A symptom of shortness of breath tends to indicate that the form of the disease is chronic.  This symptom is usually followed by a persistent cough.  For about one in fifty people the disease is eventually fatal.^{1,2,3}  To put it another way, the disease is fatal for about one in five of the people for whom sarcoidosis becomes chronic.

What are the symptoms of sarcoidosis?^{3,4}

The most usual symptoms are:

·         Tender reddish bumps or patches on the skin

·         Shortness of breath (dyspnea)

·         Cough that won’t go away

Other disease characteristics include:

·         Enlarged and tender lymph glands in the chest, around the lungs, neck, armpits, and groin

·         General feeling of being unwell physically and mentally (very similar to Chronic Fatigue Syndrome).  It may include fever, night sweats, and weight loss.

·         Painful joints.

·         Red or sore eyes.

·         Enlarged and tender lymph glands in the neck, armpits, and groin.

·         Development of abnormal heart rhythm, e.g. missed heart beats.

·         Stuffy nose.

·         Pain due to the formation of cysts (an abnormal sac-like growth) in bones.

·         Kidney stone formation.

·         Nervous system effects including hearing loss.

Any one patient is likely to present with only a few of these possible symptoms.

How is sarcoidosis diagnosed?

There is no single test that can diagnose it.  Granulomas caused by sarcoidosis are not overtly different from granulomas that occur in other diseases.  Symptoms and computer tomography scans can strongly indicate sarcoidosis, but doctors think that a definitive diagnosis requires a biopsy (excision of a small piece of skin).^{1,2,3}

How is sarcoidosis treated?

As mentioned, the disease may get better on its own over time, and many people require no treatment.  Before resorting to drugs, the following may be helpful: ^{4}

·         Quitting smoking

·         Avoiding exposure to dust, chemicals, fumes, toxic gases.

·         Eating a good diet.

·         Drinking plenty of water.

·         Taking exercise and getting plenty of sleep.

·         Some patients have high levels of calcium in their blood or urine.  For them it is advantageous to avoid excessive amounts of calcium-rich foods (such as dairy products, oranges, canned salmon with bones).  Some patients produce too much vitamin D (from their granulomas); for these patients, foods containing vitamin D should be avoided as well as too much sunlight (because it produces vitamin D).^{6}

If these conservative measures do not suffice to allow the body to cure itself, then resort has to be made to drugs.  The most effective seems to be the corticosteroid prednisone (shown to be effective in 63% of cases), but its side effects can be dire, so it is best to use it only when it becomes really necessary.^{4,7}  Other drugs are also used, with variable success, for those who cannot tolerate prednisone.^{1,2}  It has been shown that using methotrexate and prednisone together is no better than using prednisone alone.^{7}  Methotrexate can lead to liver damage.  Steroids may need to be used at low dosage for many months, sometimes a year or more.  Regular blood and lung function tests and chest x-rays are normally done during this time to check how well the treatment is working.^{3}  Clinical lung function tests do not always indicate improvements even though improvement is apparent to the patient.^{5,7}  This is because clinical lung function tests do not measure oxygen exchange.^{5}  Actual lung function may improve despite there being no change in the vital capacity of the lungs or in their ‘mechanical’ qualities (e.g. elasticity).^{5,7}

Andrew R.B. Ferguson (November 2006)

                Comments welcome, AndrewRBFerguson@hotmail.com

Note: Information for the above was drawn almost entirely from the sources shown in the references.  Note that reference 5 refers to my own experience plus personal communication with Dr John Nunn, retired anaesthetist, author of Nunn’s Applied Respiratory Physiology.  He had no knowledge of sarcoidosis, but was valuable in explaining the lacklustre changes in the three spirometry tests I had (used to clinically measure lung function) which did not reflect the changes which were manifest to me in what I was able to do in terms of the amount of breathing I needed to do in order to walk at a specified speed.

References:

1.     “Dr. Sharma's Book for Patients,” at http://www.sarcoidosissharma.com/pages/6/index.htm (checked 18 Nov 2006).  The site includes answers to 117 questions, in the Question and Answers section; and the glossary includes 102 items (though not non-caseating granulomas, which is given above!). 

2.     The Sarcoid Center, run by Dr Norman Soskel, at http://www.sarcoidcenter.com/ (checked 18 Nov 2006). 

3.     BBC page at http://www.bbc.co.uk/health/conditions/sarcoidosis1.shtml (checked 18 Nov 2006, the web page itself having been “last medically reviewed by Dr Rob Hicks in December 2005.”

4.     the Cleveland Clinic site at http://www.clevelandclinic.org/health/health-info/docs/3500/3570.asp?index=11863 (checked 18 Nov 2006).

5.     Personal communication from Dr John Nunn (see note above these references).

6.     The Marshall Protocol sites are at http://sarcinfo.com/ and http://www.marshallprotocol.com/  These provide links to papers by the outstanding team of microbiologists who are investigating Cell Wall Deficient bacteria, led by TG Marshall PhD and FE Marshall.  Many of the scientific papers are published on the web at the site of the Journal of Independent Medical Research (subtitled “collaborative publishing for the 21^stcentury”).  The protocol involves the use of angiotensin receptor blockers, ARBs (usually used in much smaller doses for reducing blood pressure), and monitoring the changes in the D hormone, so it is much more complicated than just treatment with special antibiotics (mainly minocyline).  Unless done correctly, treatment can be life threatening; and even if done correctly it causes what are known as Herxheimer reactions, that is unpleasant reactions to the toxins released by the dying bacteria.  One very big difficulty in following the Marshall Protocol is in finding a physician who knows enough about it to supervise the treatment.  At the time of writing, just how difficult that is remains to be determined.

7.     “Prednisone Improves Symptoms but not Lung Function in Sarcoidosis” (checked 18 Nov 2006).  A report presented by Alison Palkhivala to the American College of Chest Physicians, November 2001.  The link above should work.  In case it does not, the paper is on the www.docguide.com  site, but it would be difficult to locate this paper from the home page of the site. However, the prednisone paper can also be reached by using google advanced search and searching for the phrase “Prednisone improves symptoms” (the start of the title), while also specifying the domain “docguide.com.”