Sarcoidosis in Korea: Revisited

Article information

J Korean Med Assoc. 2008;51(10):925-932

Publication date (electronic) : 2008 January 10

doi : https://doi.org/10.5124/jkma.2008.51.10.925

Department of Internal Medicine, Korea University College of Medicine E – mail: keunhae@unitel.co.kr

Abstract

Sarcoidosis is a multisystem disorder of unknown origin characterized by noncaseating granulomatous inflammation. This disorder has variable clinical course, ranging from benign self-limited recovery to life-long disability. Sarcoidosis is found worldwide; however, the incidence and clinical course of the disease vary among different ethnicity and geographic regions. Especially in Korea, sarcoidosis had been known as a very rare disease until the 1st and the 2nd nationwide surveys performed in 1993 and 1998 by the Korean Academy of Tuberculosis and Respiratory Diseases. Those surveys revealed gradually increasing incidence of biopsy-proven sarcoidosis in Korea (0.027/100,000 in 1993 to 0.125/100,000 in 1998), but still very rare, and the peak age was the 30's with a female predominance (64.6%). The respiratory symptom was the most common (42%) symptom and thoracic lesion including mediastinum was the most frequently (87%) involved organ. On the other hand, cardiac involvement of this disease was very rare (0.7%). In conclusion, sarcoidosis in Korea, is a still uncommon disorder, and clinical manifestations were similar to those of western countries. Additional nationwide survey should be performed and maintained in order to investigate the correct incidence and the course of clinical manifestations of sarcoidosis in near future.

Keywords: Sarcoidosis; Incidence; Clinical manifestation; Korea

Table 1.

Clinical evaluation in sarcoidosis (adapted from N Engl J Med 2007; 357: 2153–2165)

Table 2.

Comparison of frequency of organ Involvement between united states and Korea (adapted from *; AC-CESS study and ^†; 2^nd national survey of sarcoidosis in Korea)

Table 3.

Initial therapy according to organ and clinical status (adapted from N Engl J Med 2007; 357: 2153–2165)

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Table 1.

Clinical evaluation in sarcoidosis (adapted from N Engl J Med 2007; 357: 2153–2165)

Initial Assessment

History and physical examination (attention to environmental or occupational exposure and family history)

Biopsy of affected organ, with special stains and culture of specimen

Posteroanterior and lateral chest radiographs

Pulmonary function tests – spirometry with bronchodilator, total lung capacity, and diffusion capacity

Electrocardiography

Complete ophthalmologic evaluation (slit – lamp, tonometric, and fundoscopic examinations)

Complete blood count with platelet count and measurement of serum calcium, creatine, alkaline phosphatase, alanine

aminotransferase, and aspartate aminotransferase levels)

Measurement of serum level of angiotensin – converting enzyme (if elevated, may be useful to monitor patient compliance)

Other tests as indicated for assessment of involved organs:

Heart – Holter monitoring, echocardiography, cardiac PET, MRI, and electrophysiological study for inducible arrhythmias

Lung – right-heart catheterization for pulmonary hypertension

Central nervous system – MRI with gadolinium and cerebrospinal fluid analysis

Monitoring (follow-up every 2 to 3 months)

Assessment for decline in physiological function based on initial organ involvement

Further testing in the case of new symptoms or physical findings

Testing to monitor side effects of therapy – for example, bone densitometry for corticosteroid use and semiannual ophthalmologic examination for hydroxychloroquine use

Table 2.

Comparison of frequency of organ Involvement between united states and Korea (adapted from *; AC-CESS study and ^†; 2^nd national survey of sarcoidosis in Korea)

Site of Lesion	Presentation, % in U.S*.	Presentation, % in Korea^†
Lung	95	87.1
Skin	24	30.7
Eye	12	14.2
Extrathoracic lymph node	15	12.9
Liver	12
Spleen	7
Neurologic	5	1.3
Cardiac	2	0.7

Table 3.

Initial therapy according to organ and clinical status (adapted from N Engl J Med 2007; 357: 2153–2165)

Organ	Clinical findings	Treatment
Lung	Dyspnea+FEV₁, FVC < 70% Cough, wheezing	Prednisone, 20∼40 mg/day Inhaled corticosteroid
Eye	Anterior uveitis Posterior uveitis Optic neuritis	Topical corticosteroid Prednisone, 20∼40 mg/day Prednisone, 20∼40 mg/day
Skin	Lupus pernio Plaques, nodules Erythema nodosum	Prednisone, 20∼40 mg/day Hydroxychloroquine, 400 mg/day Thalidomide, 100∼150 mg/day Methotrexate, 10∼15 mg/day Prednisone, 20∼40 mg/day Hydroxychloroquine, 400 mg/day NSAIDs
Central nervous system	Cranial nerve palsy Intracerebral involvement	Prednisone, 20∼40 mg/day Prednisone, 20∼40 mg/day Azathioprine, 150 mg/day Hydroxychloroquine, 400 mg/day
Heart	Complete heart block Ventricular fibrillation, tachycardia Decreased LVEF (< 35%)	Pacemaker AICD AICD; Prednisone, 30∼40 mg/day
Liver	Cholestatic hepatitis with constitutional symptoms	Prednisone, 20∼40 mg/day Ursodiol, 15 mg/kg/day
Joint and muscle	Arthralgias Granulomatous arthritis Myositis, myopathy	NSAIDs Prednisone, 20∼40 mg/day Prednisone, 20∼40 mg/day
Hypercalciuria hypercalcemia	Kidney stones, fatigue	Prednisone, 20∼40 mg/day Hydroxychloroquine, 400 mg/day