Sarcoidosis: Not Just a Lung Disease
Synopsis of a Presentation by Douglas Paauw, M.D.
Tenth Annual Conference on Sarcoidosis, Seattle, WA
Sarcoidosis is indeed a great puzzle. Although the subject of much research, there are no answers regarding causes, infections, genetics, diet, or the effect of the environment upon the patient. Equally, there are no definitive answers concerning the onset, duration, activity, varied manifestations, and individualistic collections of symptoms or treatment of the disease. The incidence of Sarcoidosis in the U.S. differs from other parts of the world. While Canada reports 10/100,000 cases of Sarcoidosis, Sweden has 64/100,000, US Blacks account for 35/100,000 cases and US non-Blacks comprise 10.9/ 100,000 of the cases. Worldwide, and in each population, women are the majority of Sarcoidians.
The manner by which Sarcoidosis is recognized is important, since each individual may present with different symptoms and for different reasons. Sarcoid might be identified incidentally following trauma or evaluation for an unrelated ailment. Others may seek relief of acute symptoms or an undiagnosed chronic syndrome; then the presence of non-caseating granulomas is noted on lab tests. Onset may be sudden (acute) or insidious (chronic).
The classic presentation of Sarcoidosis is hilar adenopathy (HA), (or when bilaterally, BHA), which is diagnosed by X-ray. The abnormalities are noted in the hilum area of the lungs, where all of the vessels enter the lungs and where many lymph nodes are present. Studies indicate that with the asymptomatic presentation of BHA, further tests and biopsies are not necessary to diagnose Sarcoidosis. This conclusion and practice has been supported in worldwide studies. The key word is: asymptomatic.
An acute form of skin Sarcoidosis is Lofgren’s Syndrome (LS). In such a case, the date of onset is not ambiguous. Symptoms are very noticeable and patients can recall the very painful developments. Bright red to dark red raised spots, called Erythema Nodosum (EN), are evident on the shins. BHA is noted on X-ray. Joints and lymph nodes may become swollen. A high fever, severe headache, arthralgias and arthritis contribute to the misery. Despite all this, 85% of the patients recover spontaneously from LS without long-term treatment. Duration of the various symptoms is from days to months. It has a very good prognosis. (EN is not unique to Sarcoidosis, it can occur with other disorders as well.)
Skin Sarcoidosis is quite common. Besides the previously mentioned LS and EN, other forms are Lupus Pernio (LP) and Hutchinson’s Plaque or Syndrome (HP). LP occurs more often in women, but unlike other types of Sarcoidosis, it is disfiguring. Lumpy sarcoid nodules can appear on the nose, cheeks, ears, lips and eyelids. When biopsied, the lumps note the non-caseating granulomatous cells and might be the only manifestation of Sarcoidosis.
Such a finding negates the need for other biopsies, such as in the lungs. An important factor is that 74% of individuals with LP have associated internal involvement. The distribution is approximately: intrathoracic (44%), lymph (54%), bone cysts (43%) and ocular (37%). Such involvement is not commonly seen with other forms of Sarcoidosis. LP responds poorly to the usual treatments for Sarcoidosis, namely: corticosteroids, methotrexate, hydroxychloroquine. Prednisone is essentially not effective against LP or other forms of Skin Sarcoidosis, which are difficult to treat and often require different or new treatments.
Hutchinson’s Plaque (HP) or Hutchinson’s Syndrome presents as an area of raised lumps. They are common on the back and chest and darken from red to purple. The key features are that the lumps are raised and flat on the skin’s surface. Biopsy will confirm the presence of non-caseating granulomatous tissue.
Sarcoid tissue can migrate to a pre-existing or recent scar(s) of a Sarcoidian. Such is not a unique response. Likewise, is a person whose psoriatic tissue could also enter that individual’s scar area. A biopsy of a lump in the scar tissue would definitively identify the invader.
COMMON SYMPTOMS and ISSUES in SARCOIDOSIS
Fatigue is a complex symptom in Sarcoidosis. It is usually not caused by the sarcoids. Sleep problems and depression are common causes. It must also be emphasized that medications prescribed for the treatment of Sarcoidosis, such as steroids, often play a significant role in disrupting sleep and increase the risk of depression. The latter is the most common form of fatigue. Another factor to be considered is anemia. Patients and providers should be acutely aware that fatigue has many factors.
|Onset||Often follows problem, conflict or stress in life.||Related to onset of physical ailments.|
|Duration||Chronic||Of recent onset|
|Progression||Fluctuates||Increases as disease advances.|
|Effect of Sleep||Unaffected by sleep.||Relieved by sleep – most important|
|Daily||Present in the morning, may improve during the day.||Increases as day progresses.|
KEY FEATURES of PATIENT HISTORY in WORKUP of FATIGUE
Medical texts seldom explore the important issue of chest pain with Sarcoidosis. Yet, the symptom is common with pulmonary sarcoidosis, especially when the lining of the lungs (pleura) is involved. The pains can be sharp and intermittent or sharp and stabbing. The type of pain would identify the cause; i.e. pulmonary or cardio-related. Other risk factors such as age, family history, the presence of sarcoid or some other symptom, must be considered.
CALCIUM ISSUES in PATIENTS with SARCOIDOSIS
Five to ten percent of the persons with Sarcoidosis have too much calcium in their blood (hypercalcemia); while up to fifty percent of Sarcoidians have too much calcium in their urine (hypercalciuria). The reason for such is that the granulomatous tissue deactivates the Vitamin D and the calcium is not adequately metabolized. The latter situation can lead to kidney stones. It is a well-recognized fact that the best prevention for the extremely painful potential of kidney stones, is to drink at least 1½ liters of water every day.
Because of the possibility of hypercalcimia and hypercalciuria, providers usually are cautious about advising calcium and Vitamin D supplements with the intent of fortifying bones against the ravages of osteoporosis, a serious side-effect of long-term prednisone usage. The good news is that Sarcoidians on corticosteroids have a lesser risk of having too much calcium. A second good news item is the development of the biphosphonate family of drugs for the prevention of osteoporosis and the re-building of porous bones (osteoporosis).
Sinus disease is very common in Sarcoidosis. It is usually accompanied by post-nasal drip (PND). The latter symptom also causes the “cobblestone” effect in the throat. PND leads to chronic cough and repeated clearing of the throat. Often physicians treat lungs as the probable cause of the intractable cough due to the presence of hilar adenopathy, rather than address the more common cause of the cough, PND. Treatments can occasionally include a regimen of antibiotics, saline nasal spray, and nasal steroids, with less serious side effects than steroids in pill form, for this situation.
SARCOIDOSIS and ANEMIA
Anemia occurred in 21 of 75 patients studied (28%). Nine of 17 patients who had bone marrow studies done, had non-caseating granulomas (NCG) in the marrow. This is most likely caused by the fact that the NCG crowd the marrow and decreased the production of red blood cells. Hemoglobin levels normalized in the majority of patients on steroids.