A Family Doctor’s Tale – SARCOIDOSIS

Image via Wikipedia

DOC I HAVE SARCOIDOSIS

What is Sarcoidosis?

Sarcoidosis is a disease characterized by the formation of non-caseating granulomas in a variety of tissues which usually resolve spontaneously.

Incidence is 0.01 per cent of population.
It is more common in Africans.

What causes Sarcoidosis?

The cause is unknown.

1.Most common site of involvement is the lungs with hilar lymph nodes enlargement.
2.Lymph nodes,
3.liver
4.spleen
5.skin
6.eyes
7.heart
8.kidney
9.bone marrow

Non caseating granulomatous lesions are seen in all these tissues and may be related to infection or collagen disease.
Histology showed giant cells of Langhan’s type.
Eventually the lesions go on to fibrosis and scarring

What are the symptoms of Sarcoidosis?

Sarcoidosis symptoms may be none.
Sometimes bilateral hilar enlargement are discovered only on routine chest X rays.
Intrathoracic Sarcoidosis may be divided into 4 stages:
1.Stage 0:
normal chest x rays
slight or no pulmonary function anormalities

2.Stage 1
hilar adenopathy
slight or no pulmonary function changes

3.Stage 2
hilar adenopathy
chronic intrinsic restrictive lung disease

4.Stage 3
intrinsic restrictive lung disease
obstructive lung disease
no hilar adenopathy but parenchymal infiltration

Other symptoms suggestive of sarcoidosis are:
1.erythema nodosum
2.cough
3.breathlessness
4.weight loss
5.lassitude
6.mild fever
7.superficial lymph nodes enlargement
8.enlarged liver and spleen felt on palpation
9.skin lesions
10.ocular lesions such as iridocyclitis, uveitis and retinal sarcoidosis
11.infiltrations in salivary glands, bones, joints, heart and CNS

How is the diagnosis of Sarcoidosis made?

The diagnosis of  Sarcoidosis involve
1.Biopsy and demonstration of non caseating granulomas with giant cells

2.scalene node biopsy may indicate lung disease

3.liver biopsy for forms of abdominal sarcoid

4.Kveim’s test (intradermal injection of 0.2 ml sarcoid suspension).
Mantoux test is usually negative

5.Hypercalcemia may be present

6.Angiotensin converting enzyme activity may be elevated

What are the complications for Sarcoidosis ?

1.Progression of uveitis
2.various skin joint and bone problems

What is the treatment for Sarcoidosis?

Treatment for Sarcoidosis depend on the stage:
1.Stage 0 patients do not need treatment

2.Stage 1 patients should never be treated unless there is lung function impairment

3.Stage 2 patients should be treated if pulmonary function is impaired or chest x rays reveals extensive involvement.
Observation of the condition for 3 to 6 months and treat with steroids if condition become worse

4.Stage 3 patients should be treated if the pulmonary function is affected. Corticosteroids are prescribed more freely in these patients

Corticosteroids will suppress active lesions and are particularly indicated for extra thoracic disease in a dose of 40mg to 60mg prednisolone dosage per day
for one year then slowly tapered off.
Corticosteroids also reduce the formation of fibrosis in the lungs

Local steroids are required for eye disease but systemic steroids are necessary for posterior uveitis.

What is the prognosis of Sarcoidosis?

Overall remission is 87 per cent
A clear chest x ray for 2 years means complete remission
Stage 0: very high spontaneous resolution
Stage 1: 65 per cent spontaneous remission
Stage 2: 50 per cent  spontaneous remission
Stage 3: no spontaneous remission; likely to result in respiratory failure and pulmonary hypertension

Overall mortality is 4 per cent usually from respiratory complication
Outcome is worse with systemic manifestations of disease

   Send article as PDF   

Tagged with: Chest radiograph • Conditions and Diseases • Corticosteroid • Health • Lesion • Lung • Sarcoidosis

Filed under: Medical case Studies

Like this post? Subscribe to my RSS feed and get loads more!