Friday 5 September 2014

Sarcoidosis-TB, Aspergillus, fungus balls, Candidiasis and cryptococcosis

Sarcoidosis is multisystem disease characterized by collections of inflammatory cells called granulomas. These collections are called granulomas. Granulomas occur primarily in the lungs (90%), in the skin and eye (5% to 10%), and less frequently in other body organs (Murray 1718; Yakobi).

The disease is usually temporary and self-limiting and causes no lasting damage in most individuals. Although some individuals may experience spontaneous improvement and many individuals do not require treatment, sarcoidosis can be fatal.

The cause of sarcoidosis remains unknown, but an over-reactive immune system play an important role in the disease process.Incidence and Prevalence: In the US, the incidence is about 5 cases per 100,000 in whites, and 40 cases per 100,000 in blacks (Yakobi). There is a higher incidence of disease in individuals of Scandinavian, German, Irish, Japanese, and Puerto Rican descent.



Causation and Known Risk Factors

Sarcoidosis occurs mainly in individuals between the ages of 20 to 40 years, although onset may occur at any time from childhood through old age (Yakobi). Females are more likely to develop the condition than males.



Diagnosis

History: Symptoms vary depending on the organs involved and the extent of involvement. Some individuals have no symptoms. Symptoms, when present, typically include a dry cough, shortness of breath, pain in the eye or visual disturbances, and painful joints. Occasionally individuals complain of generalized symptoms of fatigue, fever, aching muscles, or loss of appetite.Physical exam: Three physical signs often indicate sarcoidosis: enlarged nodes in the chest, a red raised rash (erythema nodosum), and inflammation in the eye (uveitis). Eye inflammation may cause redness, pain, decreased vision, and inflammatory cells and nodules within the eye. There may also be enlargement of the liver and spleen and signs specific to involvement of other organs. Involvement of the heart may be evidenced by abnormal rhythm (arrhythmia) or enlargement (cardiomyopathy). Involvement of the CNS may be evidenced by paralysis, particularly of the facial muscles (Bell's palsy).Tests: Chest x-ray may show enlarged lymph nodes and/or infiltrates of the lungs. Biopsy is often not necessary, but may be performed on tissue from the bronchus, skin, lymph nodes, conjunctivae, salivary glands, or liver. Microscopic examination of biopsied tissue typically shows the collections of inflammatory tissue (granulomas).

Blood tests typically show elevated calcium, liver enzymes, and gamma-globulin. Blood angiotensin-converting enzyme (ACE) is elevated in over half of individuals with sarcoidosis.

Pulmonary function tests may be used to detect decreased lung function. If the heart is involved, an electrocardiogram (ECG) may be performed to check heart function.



Treatment

The decision to treat depends on the individual's symptoms, the organs involved, and the extent of involvement. Individuals with disease involving the lungs, skin, liver, or joints are treated based on their symptoms. Mild symptoms typically require no treatment. Individuals with more significant symptoms or those with disease involving the eye, heart, or central nervous system do require treatment.



Corticosteroid drugs are prescribed to reduce inflammation, relieve symptoms, and prevent organ damage. Treatment is continued for up to 1 year, during which time the drug is gradually tapered or taken only every other day. If an individual cannot take corticosteroids or does not respond to the drug, a stronger immunosuppressant drug is given. A topical steroid may be added to the oral corticosteroids to treat eye involvement. Treatment specific to other organ damage is also given. The individual should follow a low-calcium, low-salt diet.

Severe fibrosis of an organ has been treated, rarely, with organ transplant. Radiation has also been used to treat localized central nervous system involvement.

When treatment is complete, the individual should be checked every 3 months for 1 to 2 years. Chest x-ray and pulmonary function tests are typically used to monitor disease activity and response to treatment.



Prognosis

The predicted outcome for sarcoidosis is variable. Generally, the prognosis is excellent. About 65% of individuals recover fully within 2 to 3 years without treatment, while individuals taking corticosteroids typically begin to see improvement within 2 to 3 weeks (Yakobi). Approximately 15% to 20% of individuals develop permanent lung damage, and about 5% eventually die from lung failure (Yakobi). Permanent lung damage is not reversible with drug therapy. Lung transplant with a matched donor lung is the only strategy that returns a healthy lung to an individual with permanent lung damage and failure. About 30% of individuals have stable, chronic disease (Murray 1727; Yakobi).



Rehabilitation

Rehabilitation of sarcoidosis needs to be modified depending on the region of the body most affected, especially in cases where the central nervous system is involved.

If the individual has a lung affected by the disease, the disorder is first treated and then followed by pulmonary rehabilitation as needed for the recovery period. Physical therapy improves ventilation of the individual experiencing sarcoidosis through the use of breathing exercises.

Once breathing improves, focus is then placed on strength and endurance by adding gradual strengthening exercises. By building endurance, the individual increases the ability to work and the resistance to fatigue. As endurance increases without shortness of breath, light upper and lower extremity exercises are initiated. Frequency of the program may vary somewhat, depending on the individual's general health.



Complications

Complications of sarcoidosis depend upon the organ affected, the extent of disease, and the time to resolution. Lesions in the muscles, heart, lungs, liver, spleen, central nervous system, or eyes may be expected to result in more complications than lesions of the skin or lymph nodes. For example, heart involvement can lead to congestive heart failure and serious arrhythmias. Eye involvement may lead to blindness, although this is rare.

Sarcoidosis and corticosteroid drugs leave a person vulnerable to certain opportunistic infections: tuberculosis, aspergillus fungus balls, candidiasis, and cryptococcosis.



Ability to Work (Return to Work Considerations)

Sarcoidosis is generally not debilitating. The individual's lungs should be protected by avoiding exposure to smoke, dust, and chemicals. Additional work restrictions depend on the location of the lesions and the extent of damage to the affected tissues or organs. If the lungs or heart are affected, appropriate work accommodations may be required until the symptoms subside. Long-term accommodations may be required if the disease relapses, persists for long periods, or causes permanent damage to essential tissues and organs.



Failure to Recover

If an individual fails to recover within the expected maximum duration period, the reader may wish to consider the following questions to better understand the specifics of an individual's medical case.Regarding diagnosis:
  • Does individual have symptoms suggestive of sarcoidosis, such as shortness of breath, persistent cough, or a rash on the face, arms or shins, eye irritation, weight loss, fatigue, night sweats, fever, and malaise?
  • Does individual have an ethnic background that puts him/her at increased risk of developing the disease?
  • Did the symptoms arise between the ages of 20 and 40 years of age?
  • Were the findings in the physical exam consistent with the characteristic findings in sarcoidosis (enlarged chest lymph nodes, rash, eye inflammation)?
  • Were other conditions with similar features ruled out?
Regarding treatment:
  • Based on the severity of symptoms, was the appropriate treatment administered? Were associated organ-specific symptoms addressed in the treatment plan? Were the corticosteroids tapered appropriately?
  • Has individual been monitored every 3 months following completion of treatment?
  • Has individual made appropriate lifestyle changes (diet, smoking cessation, etc.)?
  • Were other, more aggressive treatments (radiation therapy or organ transplant) indicated?
Regarding prognosis:
  • Has sufficient time passed since diagnosis?
  • Did individual suffer any permanent damage from advanced disease?
  • Does individual have any conditions (other lung disorders) that may complicate treatment or impact recovery?
  • Did individual suffer any opportunistic infections (tuberculosis, aspergillar fungus balls, candidiasis, and cryptococcosis) associated with the disease or corticosteroid use that affected recovery and prognosis

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