Sarcoidosis - Diagnosis and Management Summary

Epidemiology

1) High incidence in Scandinavian countries (11-24 cases per 100,000 individuals per year) and among African Americans (18-71 cases per 100,000 individuals per year)

2) Lowest in Asian countries (1 case per 100,000 individuals per year)

3) Sex: female > male (2:1)

4) Average age onset: 40-55; f 50-60y (hormonal changes around menopause) and m 30-50y

5) Global mortality: 9-14 cases per 1,000 person-years and 5-year survival is 93-95%

Pathophysiology:

1) T-cell dysfunction and increased B-cell activity → local immune hyperactivity and inflammation

2) Formation of non-caseating granulomas within the lungs and the lymphatic system

3) Fibrosis and damage of organs and tissue: Epithelioid cells → cytokines to fibroblasts → fibrosis.

4) Calcium dysregulation: activated macrophages produce 1-alpha hydroxylase → ↑ 1,25 hydroxyvitamin D (hypervitaminosis D) hyperphosphatemia, hypercalcemia, and possibly renal failure

Clinical Features:

 • Many symptoms are not caused by granulomas in a specific location but result from their release of mediators such as hypercalcaemia (~10% of patients) and/or hypercalcuria (~40%); fatigue(→ post-sarcoidosis fatigue syndrome). 30-50% patients have no symptoms at the time of diagnosis (e.g. incidental CXR finding).

 • Natural history: Self-limiting, chronic but stable or chronic and progressive

Acute sarcoidosis (1/3 of cases):  50% remits spontaneously within approx. 2 years and many others 5 years; remission less likely after 5 years. Progression to chronic sarcoidosis is rare.

 1. General: fever, malaise, lack of appetite, weight loss

 2. Pulmonary: dyspnea, cough, chest pain

 3. Extrapulmonary: ankle arthritis, anterioruveitis, erythema nodosum

 4. Otherinitial presentations: epileptic seizure, cranial neuropathy or cardiac arrest

Chronic sarcoidosis (2/3 of cases): rarely preceded by acute sarcoidosis

Pulmonary and extrapulmonary (peripheral lymph nodes: 13-15%, skin (16-32%): lupus pernio, papules, nodules; anterior/posterior MSK, heart (2-5%), liver (pain/abnormal LFT): 12-20%, spleen (pain): 5-10%, nervous system (Conductance disturbances, arrhythmias, dyspnoea, fatigue (for example, cardiomyopathy) and syncope): 2-5% Specific sarcoidosis syndromes: (distinct symptoms)

 • Löfgren syndrome: ACUTE, fever, migratory polyarthritis(symmetrical, ankles), erythema nodosum (extensor surface of legs), bilateral hilarlymphadenopathy

 • Heerfordt syndrome: Chronicclinical presentation with fever, parotitis, uveitis (iridocyclitis) and facial palsy.



Dr. Jorge Cortés @Jcortesizaguirr



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