Sarcoidosis

Sarcoidosis Prognosis Amr Badreldin Hamdy MD FCCP

One of the hallmarks of sarcoidosis is the clinical silent nature of organ involvement.

It can generally be considered as a benign disease with good prognosis.

Initial severity of symptoms and type of organ involvement may provide important prognostic information.

Sarcoidosis related mortality is low: only 1-5% of patients die from their disease, most frequently from respiratory insufficiency, neuro-sarcoidosis or cardiac involvement.

Many patients will undergo remission without specific treatment.

Pulmonary sarcoidosis

The older the age of onset, the greater the chance of chronicity.

The likelihood of spontaneous resolution is inversely correlated with the length and severity of disease:

90% of patients with Lofgren’s syndrome
65% with stage I disease
46% with stage II disease
20% with stage III disease.

Intervals and Duration of Follow-up for Sarcoidosis

Stage I disease: every 6 months.
Other stages: every 3-6 months.
Follow-up for a minimum of three years after therapy is discontinued.
If radiograph has normalized for three years, subsequent follow-up is not routinely required.

Five techniques are used in assessing the extent and severity of granulomatous inflammation and fibrosis:

1. SACE activity.
2. Gallium-67 scans.
3. BAL.
4. HRCT.
5. flurodeoxyglucose positron emission tomography.

Spirometry and F/V loops are the most useful and cost-effective parameters to follow the course of the disease. The FVC is considered the most reliable indicator of pulmonary status.
DLCO is more variable, but may detect early progressive disease, and correlates best with exercise responses

Most investigators define a change in FVC of more than 10-15% or DLCO of more than 20% as significant.
Response to therapy is usually evident within 6-12 weeks of initiation of therapy.

The presence of inflammatory markers in BAL fluid has proved disappointing as a predictor of the course of the disease (e.g. lymphocyte count, ratio of CD4 to CD8, levels of fibronectin, tumor necrosis factor alpha, interleukin-1 beta, prostaglandin E2, various cell-surface markers).

Extra-thoracic Sarcoidosis

When extra-thoracic disease is symptomatic and severe on presentation, the disease tends to be persistent and requires treatment.

Extra-thoracic disease that is asymptomatic and detected as part of an initial evaluation may not indicate a poor prognosis, since it likely reflects incidental granulomatous inflammatory disease.

Cardiac and neurosarcoidosis is associated with a ten fold increase in mortality as compared to sarcoidosis of other organs.
Uremia from chronic renal failure and hepatic failure are less common causes of death related sarcoidosis.

Forty to eighty percent of sarcoidosis deaths are secondary to pulmonary causes such as respiratory insufficiency, cor pulmonale, and massive hemoptysis.

Indications for Therapy
Absolute indications for therapy include ocular, cardiac and CNS involvement and hypercalcemia.

Pregnancy

The course of sarcoidosis appears to be improved during pregnancy and has been attributed to higher endogenous corticosteroid levels. After delivery sarcoidosis will usually return to the level of activity prior to pregnancy, although occasionally the disease can progress or worsen.

Course
Direct serial measurement of organ function provides the most important data for following the course of sarcoidosis and response to treatment.

Currently, serial clinical examination focusing on the severity of disease in affected organs remains the best approach to prognosis.

Clinical Variables Associated With Poorer Prognosis

1. Black race.
2. Onset of disease after the age of 40y.
3. Symptoms that last for more than six months.
4. Involvement of more than three organ systems.
5. Chronic uveitis.
6. Nasal mucosal sarcoidosis.

7. Central nervous system involvement.
8. Cardiac involvement.
9. Cystic bone lesions.
10. Stage III pulmonary disease.
11. Lupus pernio.
12. Hypercalcemia and nephrocalcinosis
13. Splenomegaly.

References

# Seaton A et al.: Croften and Douglas’ Respiratory Diseases. 5 th edition. Blackwell Science 2000.
# Albert R et al.: Comprehensive Respiratory Medicine. Mosby 1999.

# Goldstein RH et al.: A practical approach to Pulmonary Medicine. Lippincott-Raven 1997.
# Bordow RA et al: Manual of Clinical Problems in Pulmonary Medicine. Lippincott Williams & Wilkins. 6 th edition 2005.

# Mason RJ et al. Murray and Nadel’s Textbook of Respiratory Medicine. 4 th edition. Elsevier.Saunders 2005.
#Neuman LS et al.: Sarcoidosis. NEJM 336(17):1224.
# ATS. Statement on Sarcoidosis. Am J Respir. Crit. Care Med. 160:736 1999.

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Sarcoidosis

by scribeofegypt on Jan 17, 2008

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Sarcoidosis — Presentation Transcript