Department of Otolaryngology, Head and Neck Surgery, Bakırköy Dr. Sadi Konuk Training and Research Hospital, Istanbul, TurkeyTurkey
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Sarcoidosis is a rare systemic granulamatous disease of unknown etiology that may effect many organ and system, mainly lungs. This disease is rare in children. Sarcoidosis is staging contingent on posterior-anterior chest x-ray. Stage III rarely occurs. In generally, sarcoidosis in children is diagnosed by extrapulmonary organ involvement. We presented hereby a rare case of sarcoidosis stage III diagnosed in a child patient with acute parotitis and reviewed the relevant literature.
Keywords: Sarcoidosis; Acute parotitis; Staging; Child; Granulomatous; Chest X-ray
Sarcoidosis is a rare autoimmune systemic granulama-
and system, is characterized by a variable clinic presentation and
1,2 course.1,2 Even though it occurs more frequently at 20-40 years,
it can occur at any age. It relatively occurs rare in childhood.3 In
pediatric population, sarcoidosis is usually characterized by two
clinic presentation. One of them, it is characterized by triad of
rash, arthritis and eye involvement, occurs frequently in young
children. Other clinic presentation, older children present with
3,4 involvement of the lungs, lymph nodes and eyes as adult.3,4
Sarcoidosis may often affect lungs more than 90% of
patients, sometimes with symptoms or asymptomatic radio-
1,2 graphic abnormalities.1,2 The radiographic abnormalities con-
sist of bilateral hilar adenopathy, diffuse or local pulmonary
infiltrates, irreversible scarring and distortion. However, clinic
presentations and radiographic findings can be not similar. The
chest x-ray was categorized into four stages by Scadding, but
these stages are not necessarily denote the severity or progres-
sion of disease.3 According these stages, normal chest x-ray is
stage 0, bilateral hilar adenopathy, often with right paratracheal
adenopathy, without pulmonary infiltrates is stage 1, bilateral
hilar adenopathy with parenchymal infiltration is stage II, pa-
renchymal infiltration without hilar adenopathy is stage III and
advanced parenchymal disease demonstrating fibrosis is stage
3,4 4 4.3,4 The most common of these stages are stage 1 and 2.4 In our
case, stage III lung involvement, is rarely reported in literature
was determined according to these staging.
Sarcoidosis can affect minor and major salivary glands,
5-8 mainly parotid gland, approximately 6% of patients.5-8 Parotid
gland affecting cause variable clinic presentation between acute
parotitis and diffuse parotid enlargement due to chronic inflam-
mation. It can cause sicca like-symptom in patients. In our case,
acute parotitis was determined but there were no any sicca like-
symptoms as extrapulmonary involvement. In literature, this
clinic presentation was reported in rare.1
Sarcoidosis is diagnosed by a correlation between clini-
cal and radiographic findings supported by histopathological ex-
amination. However, there are no specific laboratory tests for
diagnosis of sarcoidosis but some abnormalities in laboratory
tests can be determined such as elevated erythrocyte sedimenta-
tion rate and other acute phase reactants, anemia, leukopenia and
1-3 hypergammaglobulinemia.1-3 Even though serum ACE levels are
elevated in more 40% patients with sarcoidosis, the value of a
1,9 serum ACE level for diagnosis of sarcoidosis remains limited.1,9
In our case, abnormalities in laboratory tests were elevated ACE
and acute phase reactants.
The differential diagnosis of sarcoidosis consist of
granulamatous disease such as tuberculosis, lymphoma, para- 3,4,10 sitic infections.
The treatment of sarcoidosis depends on its severity
but typically includes prednisolone and other immunosuppres-
sive drugs, especially methotrexate. However, medical therapy
is not mandated for sarcoidosis because of regression spontane-
3,6,8
kg/day in a 2 years and the severity of his symptoms decreased
following treatment, prednisolone dose was reduced to 2 mg/
day.
Sarcoidosis should be considered in the differential di-
agnosis of patients with acute parotitis in children. A high de-
gree of clinical suspicion is needed to determine this rare po-
tential etiology. Because patients with sarcoidosis can present
with many different manifestations affecting multiple systems,
the otolaryngologist must be aware of sarcoidosis and its rare
presentations.
Written informed consent was obtained from the patient de-
scribed in this case.
The authors declare that they have no conflicts of interest.
The authors declare that this case has received no financial sup-
port.