Sarcoidosis

04/12/2023 Sarcoidosis Prof. Dr. Saad S Al Ani Khorfakkan .Sharjah ,UAE

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SarcoidosisProf. Dr. Saad S Al Ani

Senior Pediatric Consultant

Head of Pediatric Department

Khorfakkan Hospital, Sharjah ,UAE

[email protected]

Sarcoidosis• A chronic multisystem granulomatous

disease of unknown cause • Occurs most frequently in young adults but

can occur during childhood.

04/12/2023 2Sarcoidosis Prof. Dr. Saad S Al Ani

Khorfakkan .Sharjah ,UAE

Etiology • The etiology remains obscure.• ? alteration to the immune response after

exposure to an environmental, occupational, or infectious agent



Epidemiology•Involving all ethnic groups • Familial clustering of this disease has been observed •? genetic predisposition; •Mode of inheritance is unclear



Cont.•The noncaseating granuloma contain: * Epithelioid cells * Macrophages * Giant cells in the center Surrounded by a mixture of : * Monocytes * Lymphocytes *Fibroblasts.



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



Cont.•During active disease, lymphocytes in the granulomas are predominantly helper T (CD4) lymphocytes. •These lesions usually heal with complete preservation of the parenchyma



Cont.•In approximately 20% of the lesions, fibroblasts proliferate at the periphery of the granuloma and may produce fibrotic scar tissue



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.



Signs and symptoms of sarcoidosis•

Signs and symptoms of sarcoidosis



•

Few small follicles in the inferior palpebral conjunctiva of the right eye with sarcoidosis



Pulmonary involvement * Parenchymal infiltrates * Miliary nodules * Hilar and paratracheal lymphadenopathy



Chest x ray showing the typical nodularity of sarcoidosis in the base of the lungs.

http://www.labtestsonline.org/understanding/analytes/ac



http://www.labtestsonline.org/understanding/analytes/ac

Sarcoidosis in a white 10-yr-old girlThere are:1.Widely disseminated peribronchial infiltrations 2.Multiple small nodular densities 3.Hyperaeration of the lungs 4.Hilar lymphadenopathy.



CT scan of the chest •

lymphadenopathy (arrows) in mediastinum due to sarcoidosis



http://en.wikipedia.org/wiki/Computed_tomography

http://en.wikipedia.org/wiki/Lymphadenopathy

http://en.wikipedia.org/wiki/Mediastinum

Cont.•Pulmonary function tests show restrictive changes. •Peripheral lymphadenopathy• Eye changes consisting of uveitis or iritis• Skin lesions• Hepatic involvement



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.



Diagnosis •There are no specific diagnostic tests. *↑erythrocyte sedimentation rate * Hyperproteinemia * Hypercalcemia * Hypercalciuria * Eosinophilia * ↑ angiotensin-converting enzyme level are common



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 in a lymph node



Asteroid body in sarcoidosis

04/12/2023 21Sarcoidosis Prof. Dr. Saad S Al Ani Khorfakkan .Sharjah ,UAE

http://en.wikipedia.org/wiki/Asteroid_body

Micrograph showing pulmonary sarcoidosis with granulomas with

asteroid bodies, H&E stain



http://en.wikipedia.org/wiki/Micrograph

http://en.wikipedia.org/wiki/Pulmonary

http://en.wikipedia.org/wiki/Granuloma

http://en.wikipedia.org/wiki/Asteroid_bodies

http://en.wikipedia.org/wiki/H&E_stain

Gross pathology image Sarcoidosis with honeycombing: Prominent honeycombing is present in the lower lobes accompanied by fibrosis and some honeycombing in the upper lungs.



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.



Differential Diagnosis Includes: * Tuberculosis * Pulmonary mycoses (histoplasmosis, blastomycosis, and coccidioidomycosis) * Lymphoma * Crohn disease * Inflammatory ocular lesions such as phlyctenular conjunctivitis



Treatment•Treatment is symptomatic and supportive•Corticosteroids may suppress the acute manifestations, especially the: *Inflammatory ocular lesions *Progressive pulmonary disease *Hypercalcemia/hypercalciuria.




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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.

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.

04/12/2023 28Sarcoidosis Prof. Dr. Saad S Al Ani Khorfakkan .Sharjah ,UAE

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.



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): 1969–71.



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.



http://www.nhlbi.nih.gov/health/dci/Diseases/sarc/sar_signsandsymptoms.html

http://www.nhlbi.nih.gov/health/dci/Diseases/sarc/sar_signsandsymptoms.html

http://www.aaimedicine.org/journal-of-insurance-medicine/jim/2004/036-01-0091.pdf


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Sarcoidosis

Health & Medicine

pulmonary sarcoidosis

unclear sarcoidosis

parenchyma sarcoidosis

sarcoidosis granulomasproduce

sarcoidosis shoul

distinct form of sarcoidosis

pulmonary changes

significant eye disease