Sarcoidosis
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Transcript of 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
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
04/12/2023 3Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Epidemiology•Involving all ethnic groups • Familial clustering of this disease has been observed •? genetic predisposition; •Mode of inheritance is unclear
04/12/2023 4Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Cont.•The noncaseating granuloma contain: * Epithelioid cells * Macrophages * Giant cells in the center Surrounded by a mixture of : * Monocytes * Lymphocytes *Fibroblasts.
04/12/2023 5Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
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
04/12/2023 6Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Cont.•During active disease, lymphocytes in the granulomas are predominantly helper T (CD4) lymphocytes. •These lesions usually heal with complete preservation of the parenchyma
04/12/2023 7Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Cont.•In approximately 20% of the lesions, fibroblasts proliferate at the periphery of the granuloma and may produce fibrotic scar tissue
04/12/2023 8Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
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.
04/12/2023 9Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Signs and symptoms of sarcoidosis•
Signs and symptoms of sarcoidosis
04/12/2023 10Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
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Few small follicles in the inferior palpebral conjunctiva of the right eye with sarcoidosis
04/12/2023 11Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Pulmonary involvement * Parenchymal infiltrates * Miliary nodules * Hilar and paratracheal lymphadenopathy
04/12/2023 12Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Chest x ray showing the typical nodularity of sarcoidosis in the base of the lungs.
http://www.labtestsonline.org/understanding/analytes/ac
04/12/2023 13Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
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.
04/12/2023 14Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
CT scan of the chest •
lymphadenopathy (arrows) in mediastinum due to sarcoidosis
04/12/2023 15Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Cont.•Pulmonary function tests show restrictive changes. •Peripheral lymphadenopathy• Eye changes consisting of uveitis or iritis• Skin lesions• Hepatic involvement
04/12/2023 16Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
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.
04/12/2023 17Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Diagnosis •There are no specific diagnostic tests. *↑erythrocyte sedimentation rate * Hyperproteinemia * Hypercalcemia * Hypercalciuria * Eosinophilia * ↑ angiotensin-converting enzyme level are common
04/12/2023 18Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
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.
04/12/2023 19Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Sarcoidosis in a lymph node
04/12/2023 20Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Asteroid body in sarcoidosis
04/12/2023 21Sarcoidosis Prof. Dr. Saad S Al Ani Khorfakkan .Sharjah ,UAE
Micrograph showing pulmonary sarcoidosis with granulomas with
asteroid bodies, H&E stain
04/12/2023 22Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Gross pathology image Sarcoidosis with honeycombing: Prominent honeycombing is present in the lower lobes accompanied by fibrosis and some honeycombing in the upper lungs.
04/12/2023 23Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
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.
04/12/2023 24Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Differential Diagnosis Includes: * Tuberculosis * Pulmonary mycoses (histoplasmosis, blastomycosis, and coccidioidomycosis) * Lymphoma * Crohn disease * Inflammatory ocular lesions such as phlyctenular conjunctivitis
04/12/2023 25Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
Treatment•Treatment is symptomatic and supportive•Corticosteroids may suppress the acute manifestations, especially the: *Inflammatory ocular lesions *Progressive pulmonary disease *Hypercalcemia/hypercalciuria.
04/12/2023 26Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
04/12/2023 Sarcoidosis Prof. Dr. Saad S Al Ani Khorfakkan .Sharjah ,UAE
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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.
04/12/2023 2929Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
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.
04/12/2023 30Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
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.
04/12/2023 31Sarcoidosis Prof. Dr. Saad S Al Ani
Khorfakkan .Sharjah ,UAE
04/12/2023 Sarcoidosis Prof. Dr. Saad S Al Ani Khorfakkan .Sharjah ,UAE
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