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Sarcoidosis

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

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

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  • 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 9 Khorfakkan .Sharjah ,UAE
  • 10. Signs and symptomsof sarcoidosisSigns and symptoms of sarcoidosis • Sarcoidosis Prof. Dr. Saad S Al Ani12/7/2012 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 Ani12/7/2012 11 Khorfakkan .Sharjah ,UAE
  • 12. Pulmonary involvement * Parenchymal infiltrates * Miliary nodules * Hilar and paratracheal lymphadenopathy Sarcoidosis Prof. Dr. Saad S Al Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 14 Khorfakkan .Sharjah ,UAE
  • 15. CT scan of the chest •lymphadenopathy (arrows) in mediastinum dueto sarcoidosis Sarcoidosis Prof. Dr. Saad S Al Ani12/7/2012 15 Khorfakkan .Sharjah ,UAE
  • 16. Cont. •Pulmonary function tests show restrictive changes. •Peripheral lymphadenopathy • Eye changes consisting of uveitis or iritis • Skin lesions • Hepatic involvement12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 19 Khorfakkan .Sharjah ,UAE
  • 20. Sarcoidosis in a lymph node Sarcoidosis Prof. Dr. Saad S Al Ani12/7/2012 20 Khorfakkan .Sharjah ,UAE
  • 21. Asteroid body in sarcoidosis Sarcoidosis Prof. Dr. Saad S Al Ani12/7/2012 21 Khorfakkan .Sharjah ,UAE
  • 22. Micrograph showing pulmonary sarcoidosis with granulomas witha steroid bodies, H&E stain Sarcoidosis Prof. Dr. Saad S Al Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 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 Ani12/7/2012 31 Khorfakkan .Sharjah ,UAE
  • 32. Sarcoidosis Prof. Dr. Saad S Al Ani12/7/2012 32 Khorfakkan .Sharjah ,UAE

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