Sarcoidosis

 

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Original Editors - Jordan Bormann from Bellarmine University's Pathophysiology of Complex Patient Problems project.

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Definition/Description

Sarcoidosis (sar-coy-doe-sis) is a potentially life threatening disease with no known cause or cure. The duration of this disease varies and can last from only a few short months to several years. Often times, sarcoidosis is self-limiting needing no medicinal treatment. The primary manifestation of sarcoidosis includes inflammation of the lungs as well as many other bodily organs. About 90% of diagnosed cases are deemed to be pulmonary sarcoidosis. The pulmonary involvement affects the lungs by forming inflamed masses of tissue known as granulomas in the lung parenchyma (alveoli, bronchioles) and lymph nodes^,2. Results include stiffness of the lungs due to scar tissue formation causing decreased oxygen uptake and gas exchange in the bloodstream². Other involvement of sarcoidosis can manifest itself as inflammation of the epithelial tissue, eyes, liver, spleen, heart, kidneys, central nervous system (CNS) and bones of the hands or feet¹. Although this disease has not always been well known, cases have been recorded worldwide and are becoming increasingly more common³.

Prevalence

Patients most commonly affected by sarcoidosis are 20-40 years of age¹.  Women are more affected than men (6.3 per 100,000; 5.9 per 100,000) listed respectively.  African Americans are the most diagnosed ethnic group in the United States and have a prevalence of four times that of the diagnosis for Caucasians (2.4%; 0.85%)⁴.  In the United Kingdom, 5-10 per 100,000 individuals are diagnosed with sarcoidosis.  It is the most common interstitial lung disorder in the western part of the world and has an increase incidence in Irish, West Indian, and Scandinavian people⁵.

Nowack et al. BMC Dermatology 2002 2:15 doi:10.1186/1471-5945-2-15 http://www.biomedcentral.com/1471-5945/2/15

Nowack et al. BMC Dermatology 2002 2:15 doi:10.1186/1471-5945-2-15 http://www.biomedcentral.com/1471-5945/2/15

Characteristics/Clinical Presentation

   

Photo Courtesy of BMC Dermatology

Available at http://wapedia.mobi/en/Sarcoidosis

Associated Co-morbidities

As with many other systemic diseases, sarcoidosis has several co-morbidities.  Of all the patients diagnosed, 10-35% will suffer from acute arthritic changes.  These symptoms most commonly occur in the knees and ankles symmetrically and involves many joints.  The arthritic changes vary in their duration and are self-limiting within weeks to months.  It is possible that these symptoms may become chronic and reoccur for periods of time, but joint changes continue to stay minimal.  These symptoms vary in their timing of the disease and may become present early or late in the disease progression.  Along with the arthritis, erythema nodosum and hilar adenopathy may also be present.  These three symptoms combined are known as Lofgren's syndrome and can also be self-limiting.  Muscle involvement may also present itself as sarcoid myositis.  Other co-morbidities include nerve palsies, mononeuropathy, and polyneuropathy.  Polyneuropathy is more rare than other symptoms and may present symmetrically¹.

Medications

The most common type of medication used for treating sarcoidosis is corticosteroids.  There are two main steroidal medications used which include prednisone and prednisolone4.  Antimalarial agents may also be used for treatment.  Integumentary symptoms and hypercalcemia are often treated with the antimalarials chloroquine (aralen) or hydrochloroquine (plaquenil) and other drugs such as thalidomide (thalomid).  For more chronic symptoms of sarcoidosis, methotrexate (rheumatrex) is used although it is ineffective for six months or longer.  Azathioprene (imuran) has several common side effects of including nausea and neutropenia.  Other drugs are limited in their effects due to toxicity levels such as cyclophosphamide (cytoxan).  Although many other drugs are available and have been tested, several do not have evidence based date or reports of their usefulness.  Such drugs include pentoxifyline (trental), cycloporine (neoral), and infliximab (remicade)⁴.

Diagnostic Tests/Lab Tests/Lab Values

Sarcoidosis is often time hard to diagnose due to no specific testing for the diagnostic process.  It is most commonly diagnosed through a clinical examination and thorough history¹.  Additional testing methods for the evaluation and diagnosis of sarcoidosis include posteroanterior chest radiographs, pulmonary functioning tests (spirometry, lung capacity),  blood counts (WBCs, RBCs, platelets), serum levels (calcium, liver enzymes, creatine, urea nitrogen), and tissue biopsy (lymph nodes, skin, salivary glands, transbronchial)^1,4.  Other include urine analysis, electrocardiogram (ECG), opthalmologic examination, and Tuberculin skin test⁴.  Several other granulomatous diseases must be ruled out before a diagnosis of sarcoidosis is given.  These disease include tuberculosis, lymphoma, carcinoma, berylliosis, and several fungal diseases¹.  Of the symptoms present, CNS and pulmonary involvement are usually diagnosed on a delayed time period^1,4.  However, dermatological symptoms are most easily diagnosed⁴.

Etiology/Causes

Sarcoidosis has no known cause.  However, some researchers believe the course of the disease may have genetic connections or result from an immune response⁶.  Others possible causes include viruses, bacterias, dust particles, or chemical exposure⁷.  Due to the vast nature of the manifestation, other researchers have led one to believe that there may be several causes¹.

Systemic Involvement

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Medical Management (current best evidence)

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Physical Therapy Management (current best evidence)

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Alternative/Holistic Management (current best evidence)

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Differential Diagnosis

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Case Reports/ Case Studies

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Resources

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Recent Related Research (from Pubmed)

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References

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1. Fuller KS, Goodman CC. Pathology implications for the physical therapist. 3rd ed. St. Louis: Saunders, 2009.

2. American Lung Association. Lung Diseases: sarcoidosis. http://www.lungusa.org/lung-disease/sarcoidosis/ (Accessed March 16,2011)

3. Foundation for Sarcoidosis research. Sarcoidosis: General Info, Symptoms, Diagnosis, Treatment. http://www.stopsarcoidosis.org/sarcoidosis/diseasefacts.htm (Accessed March 16, 2011)

4. Wu JJ, Schiff KR. Sarcoidosis. American Family Physician. 2004; 70,2: 312-322.

5.  Ho LP, Urban BC, Thickett DR, Davies RJ. Deficiency of a subset of T cells with immunoregulatory properties in sarcoidosis. The Lancet. 2005; 365: 1062-1072. <o:p></o:p>