Definition ,presentation ,diagnosis,differential diagnosis ,treatment ,prognosis

1. Sarcoidosis
Prof. Dr. Saad S Al Ani
Senior Pediatric Consultant
Head of Pediatric Department
Khorfakkan Hospital, Sharjah ,UAE
saadsalani@yahoo.com
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 1
Khorfakkan .Sharjah ,UAE

2. Sarcoidosis
• A chronic multisystem granulomatous
disease of unknown cause
• Occurs most frequently in young adults but
can occur during childhood.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 2
Khorfakkan .Sharjah ,UAE

3. Etiology
• The etiology remains obscure.
• ? alteration to the immune response after
exposure to an
environmental, occupational, or infectious
agent
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 3
Khorfakkan .Sharjah ,UAE

4. Epidemiology
•Involving all ethnic groups
• Familial clustering of this disease has been
observed
•? genetic predisposition;
•Mode of inheritance is unclear
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 4
Khorfakkan .Sharjah ,UAE

5. Cont.
•The noncaseating granuloma contain:
* Epithelioid cells * Macrophages
* Giant cells
in the center
Surrounded by a mixture of :
* Monocytes * Lymphocytes
*Fibroblasts.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 5
Khorfakkan .Sharjah ,UAE

6. Cont.
•Activated lymphocytes and macrophages
within the granulomas release various
mediators including :
* Interleukin-1 (IL-1), IL-2
* Interferon
* Other cytokines
to promote and maintain granulomatous
lesions
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 6
Khorfakkan .Sharjah ,UAE

7. Cont.
•During active disease, lymphocytes in the
granulomas are predominantly helper T
(CD4) lymphocytes.
•These lesions usually heal with complete
preservation of the parenchyma
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 7
Khorfakkan .Sharjah ,UAE

8. Cont.
•In approximately 20% of the
lesions, fibroblasts proliferate at the
periphery of the granuloma and may
produce fibrotic scar tissue
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 8
Khorfakkan .Sharjah ,UAE

9. Cont.
•Macrophages within sarcoidosis granulomas
produce and secrete 1,25-(OH)2-D3,
•Excess vitamin D results in hypercalcemia
and hypercalciuria in patients with
sarcoidosis.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 9
Khorfakkan .Sharjah ,UAE

10. Signs and symptomsof sarcoidosis
Signs and symptoms of sarcoidosis
•
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 10
Khorfakkan .Sharjah ,UAE

11. Few small follicles in
•
the inferior palpebral
conjunctiva of the
right eye with
sarcoidosis
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 11
Khorfakkan .Sharjah ,UAE

12. Pulmonary involvement
* Parenchymal infiltrates
* Miliary nodules
* Hilar and paratracheal
lymphadenopathy
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 12
Khorfakkan .Sharjah ,UAE

13. Chest x ray showing the typical nodularity of
sarcoidosis in the base of the lungs.
http://www.labtestsonline.org/understanding/analytes/ac
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 13
Khorfakkan .Sharjah ,UAE

14. Sarcoidosis in a white 10-yr-old girl
There are:
1.Widely disseminated
peribronchial infiltrations
2.Multiple small nodular
densities
3.Hyperaeration of the lungs
4.Hilar lymphadenopathy.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 14
Khorfakkan .Sharjah ,UAE

15. CT scan of the chest
•
lymphadenopathy (arrows) in mediastinum due
to sarcoidosis
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 15
Khorfakkan .Sharjah ,UAE

16. Cont.
•Pulmonary function tests show
restrictive changes.
•Peripheral lymphadenopathy
• Eye changes consisting of uveitis or
iritis
• Skin lesions
• Hepatic involvement
12/7/2012 Sarcoidosis Prof. Dr. Saad S Al Ani
16
Khorfakkan .Sharjah ,UAE

17. Cont.
•Children younger than 4 yr old may have a
distinct form of sarcoidosis consisting of:
1. Maculopapular erythematosus rash
2.Uveitis
3.Arthritis (large, painless, boggy synovial
effusions of the tendon sheaths with little
limitation of motion.)
 Minimal to no pulmonary changes.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 17
Khorfakkan .Sharjah ,UAE

18. Diagnosis
•There are no specific diagnostic tests.
*↑erythrocyte sedimentation rate
* Hyperproteinemia
* Hypercalcemia
* Hypercalciuria
* Eosinophilia
* ↑ angiotensin-converting enzyme
level
are common
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 18
Khorfakkan .Sharjah ,UAE

19. Kveim test
* Consisting of intradermal injection of
material from a sarcoid lesion and
observation for the formation of a
granuloma several weeks later
* Is used infrequently because of the
difficulty in obtaining standardized test
material and reports of varying
sensitivity and specificity of the test.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 19
Khorfakkan .Sharjah ,UAE

20. Sarcoidosis in
a lymph node
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 20
Khorfakkan .Sharjah ,UAE

21. Asteroid body
in sarcoidosis
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 21
Khorfakkan .Sharjah ,UAE

22. Micrograph showing
pulmonary sarcoidosis
with granulomas witha
steroid bodies, H&E
stain
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 22
Khorfakkan .Sharjah ,UAE

23. Gross pathology image
Sarcoidosis with
honeycombing:
Prominent
honeycombing is present
in the lower lobes
accompanied by fibrosis
and some honeycombing
in the upper lungs.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 23
Khorfakkan .Sharjah ,UAE

24. Cont.
• Significant eye disease and renal damage
from hypercalciuria can occur without
symptoms
•All patients with sarcoidosis should be
evaluated at the initial presentation and
monitored at regular intervals for evidence
of ocular disease and hypercalciuria.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 24
Khorfakkan .Sharjah ,UAE

25. Differential Diagnosis
Includes:
* Tuberculosis
* Pulmonary mycoses (histoplasmosis,
blastomycosis, and coccidioidomycosis)
* Lymphoma
* Crohn disease
* Inflammatory ocular lesions such as
phlyctenular conjunctivitis
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 25
Khorfakkan .Sharjah ,UAE

26. Treatment
•Treatment is symptomatic and supportive
•Corticosteroids may suppress the acute
manifestations, especially the:
*Inflammatory ocular lesions
*Progressive pulmonary disease
*Hypercalcemia/hypercalciuria.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 26
Khorfakkan .Sharjah ,UAE

27. Cont.
•Methotrexate may be considered in severe
cases that are unresponsive to corticosteroid
therapy
•Eye involvement may lead to blindness;
therefore, therapy with topical corticosteroids
with careful monitoring is warranted.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 27
Khorfakkan .Sharjah ,UAE

28. Prognosis
•Spontaneous recovery may occur after a
prolonged illness of several months to
several years, or the condition may be
chronic, with progressive lung disease
•Pulmonary function tests are useful in
following the progress of lung
involvement
•Angiotensin-converting enzyme levels
have been shown to correlate with disease
activity.
12/7/2012 Sarcoidosis Prof. Dr. Saad S Al Ani 28
Khorfakkan .Sharjah ,UAE

29. References
•Polito C, LaManna A, Cioce F, et al:
Clinical presentation and natural
course of idiopathic hypercalciuria in
children. Pediatr Nephrol 2000;
15:211-14.
•Vachvanichsanong P, Malagon
M, Moore ES: Recurrent abdominal
and flank pain in children with
idiopathic hypercalciuria. Acta
Paediatr 2001; 90:643-48.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 2929
Khorfakkan .Sharjah ,UAE

30. Cont.
• Gandhi N, Oetting TA, Kirby P:
Ocular Sardoidosis: A systems-based
approach to diagnosis and treatment.
EyeRounds.org. November 5, 2007;
•Verschueren K, Van Essche
E, Verschueren P, Taelman
V, Westhovens R (November 2007).
"Development of sarcoidosis in
etanercept-treated rheumatoid arthritis
patients". Clin. Rheumatol. 26 (11):
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 1969–71. Khorfakkan .Sharjah ,UAE 30

31. Cont.
•Stokes MB, Foster K, Markowitz GS (July 2005).
"Development of glomerulonephritis during anti-
TNF-alpha therapy for rheumatoid
arthritis". Nephrol. Dial. Transplant. 20 (7): 1400–6.
•National Heart, Lung, and Blood Institute: DCI
Home: Lung Diseases: Sarcoidosis: Signs &
SymptomsRetrieved on May 9, 2009
•Joanne Mambretti (2004). "Chest X-ray Stages of
Sarcoidosis". Journal of Insurance Medicine: 91-92.
Retrieved June 3, 2012.
Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 31
Khorfakkan .Sharjah ,UAE

32. Sarcoidosis Prof. Dr. Saad S Al Ani
12/7/2012 32
Khorfakkan .Sharjah ,UAE