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Sarcoidosis

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What is Sarcoidosis ?

It is a multi-systemic disease which occurs as small collections of inflammatory cells called granulomas being deposited anywhere in the body.

It most commonly occurs in the lungs and the lymph nodes. It is also seen in eyes, brain, skin,heart , liver and other organs.

How does it all happen?

We can describe the changes taking place in three stages.

• First being the inflammatory stage. The immune system is activated and there is increase in number of B- lymphocytes.

• Second stage is where formation of multiple tiny lump of inflammatory cells/ tissue (granulomas) takes place in response to the immune fight back mechanism and these are a classic sign for sarcoidosis.

• The third stage and final stage is scarring (fibrosis) of tissues and the organs. It may prove to be fatal at times.

In case of lung, there is distribution of the granulomas along the lymphatic in the bronchovascular sheath and also along the interlobular septa & subpleural region of the lung in some cases.

In some patients it may occur that as the disease progresses from one stage to another, it spreads from one tissue/organ to another, while in few of the patients it may remain restricted to the same organ

In few cases it has seen to go unnoticed by the patient and the granulomas resolve on their own.

In few patients it has shown to be fatal and progress to irreversible fibrosis.

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Causes:

Environmental antigens

Genetics

Unknown cause

Risk factors:

• Age: the disease occurs generally between 20-40 years of age

• Gender: More common in females than males.

• Genetics: plays an important role in some conditions/ causes.African-american> Caucasians

Symptoms:

Generalised symptoms

• Weight loss

• Fatigue

• Swollen lymph nodes

• Pain and swelling in joints

Lung symptoms

• Persistent dry cough

• Shortness of breath

• Wheezing

• Chest pain

Other organ symptoms

• A rash of red or reddish-purple bumps which are generally seen around the shins or ankles, they may be warm and tender on touch.

• Disfiguring sores (lesions) on the nose, cheeks and ears

• Areas of skin that are darker or lighter in color

• Growths under the skin (nodules), particularly around scars or tattoos

• Blurred vision

• Eye pain

• Burning, itching or dry eyes

• Severe redness

• Sensitivity to light

• Chest pain

• Shortness of breath (dyspnea)

• Fainting (syncope)

• Fatigue

• Irregular heartbeats (arrhythmias)

• Rapid or fluttering heart beat (palpitations)

• Swelling caused by excess fluid (edema)

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Complications:

• Pulmonary hypertension results due to damage to the pulmonary blood vessels.

• Heart failure is a serious complication that occurs due to inflammation resulting in burdening the heart which fails to pump enough blood.

• Pulmonary fibrosis

• Pulmonary embolism

• Aspergillomas complicated by hemoptysis.

Diagnosis:

Lung function test – Spirometer devices are used to assess the pulmonary functioning in form of how long the lung can hold , take in and move out the air.It is helpful in assessment of the the on going treatment and in grading the seriousness the disease.It helps in characterization of physiological impairment and knowing diffusion capacity for carbon monoxide in cases with lung fibrosis.

Treatment:

• Corticosteroids

• Use of immunosuppressant therapy helps by reducing the inflammatory response.

Prognosis

• stage 1 : 60 % resolution within 1-2 years

• stage 2 show 46 % of prognosis rate

• Last stage 3 shows prognosis of jus 12 %

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