Sarcoidosis

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Sarcoidosis

  1. 1. Sarcoidosis<br />
  2. 2. Sarcoidosis<br /><ul><li>Sarcoidosis is a multisystem disorder characterized by the presence of noncaseatinggranulomas.</li></ul>Most affected organ is the lung<br />Skin, eyes and lymph nodes are frequently involved<br />Acute or sub acute and self limiting<br />Waxing and waning over years<br />
  3. 3. Incidence and Prevalence<br /><ul><li>Disease can affect people of any age, race and gender.
  4. 4. 20 to 40 years of age.
  5. 5. Most cases are Sporadic . 5 % of patients – FH of Sarcoidosis.</li></li></ul><li>Pathophysiology<br />Granulomas are Pathologic hall mark of sarcoidosis.<br />Granuloma is an organized collection of macrophages.<br />
  6. 6. granulomas form in response to antigens that are resistant to "first-responder" inflammatory cells such as neutrophils and eosinophils.<br />The antigen causing the formation of a granuloma is most often an infectious pathogen or environmental agent, but often the offending antigen is unknown<br />
  7. 7. The antigenic triggering agents cause activation of the Helper T-cell and Macrophages <br /><ul><li>Activated Helper T-cell and Macrophages produce IL-2,IFN and TNF complex interaction of cytokines Inflammatory response leading to granuloma</li></li></ul><li><ul><li>Giant cells in the central part of the granuloma.
  8. 8. Giant cells are fused Macrophages-Langerhans giant cells. </li></ul>The central epithelioid and giant cells are surrounded by a rim of lymphocytes, mostly T-helper cells<br />
  9. 9. All granulomas, regardless of cause, may contain additional cells and matrix. These include lymphocytes, neutrophils, eosinophils, fibroblasts and collagen [fibrosis].<br />
  10. 10. CYTOPLASMIC INCLUSION BODY Schaumann body (arrow) is common in sarcoidosis but is nonspecific.<br />
  11. 11. Clinical Manifestations<br />50% patients are asymptomatic<br />Abnormal "routine" chest radiograph<br />Symptomatic patients, with wide variety of symptoms<br />Onset is usually insidious but can be acute<br />
  12. 12. Clinical Manifestations<br />Respiratory symptoms are most common<br />Cough, chest discomfort, and dyspnea <br />Symptoms reflect the specific organs involved by the granulomas<br />
  13. 13. Lungs<br />First site involved<br />Begins with alveolitis involving small bronchi and small blood vessels <br />Alveolitis either clears up spontaneously or leads to granuloma <br />Fibrosis<br />
  14. 14. Noncaseating granuloma in lung is the characteristic lesion of sarcoidosis.<br />
  15. 15. CASEOUS NECROSISCellular destruction in TB granuloma appears as clumped debris (arrows). This necrosis does not occur in sarcoidosis.<br />
  16. 16. M. tuberculosis BACILLI Caseous necrosis is most common in TB, but Gram negative, acid fast bacilli must be identified to make the diagnosis.<br />
  17. 17. SUBPLEURAL GRANULOMA IN LUNG<br />
  18. 18. Eyes<br />25% have eye lesions<br />Blurred vision, pain, photophobia and dry eyes<br />Chronic uveitis leads to glaucoma, cataracts and blindness <br />Keratoconjunctivitis sicca<br />Papilledema <br />
  19. 19. CONJUNCTIVITIS<br />
  20. 20. PAPILLEDEMA Often associated with 7th nerve facial palsy.<br />
  21. 21. Skin<br />33% have skin lesions<br />LOFGREN'S SYNDROME; acute triad of erythemanodosum, joint pains, and bilateral hilaradenopathy<br />
  22. 22. NAKED GRANULOMA Young granulomas (arrows) in the skin with no surrounding rim of mononuclear cells.<br />
  23. 23. ERYTHEMA NODOSUM These reddish raised lesions. <br />
  24. 24. Skin<br />Lupus pernio- indurated blue purple swollen shiny lesions on nose, cheeks, lips, ears and fingers. <br />Papules, nodules, and plaques <br />Psoriatic like lesions <br />
  25. 25. LUPUS PERNIO <br />
  26. 26. RAISED PLAQUESThese raised plaques are the result of coalescence of nodules.<br />
  27. 27. PSORIASIS LIKE LESIONS These small white lesions closely resemble psoriasis.<br />
  28. 28. Liver<br />33% have hepatomegaly or biochemical evidence of disease <br />Symptoms usually absent<br />Cholestasis, fibrosis, cirrhosis, portal hypertension, and the Budd-Chiari syndrome have been seen <br />
  29. 29. Musculoskeletal<br />Acute polyarthritis with fever is common<br />Arthritis is self limited<br />Chronic destructive bone disease with deformity is rare<br />Muscle disease is rare<br />
  30. 30. PUNCHED OUT LYTIC LESIONS Focal osteolytic lesions in the fingers are most common abnormality.<br />
  31. 31. LACY TRABECULAR PATTERN Osteolysis has left a lacy trabecular pattern in this phalanx (arrow)<br />
  32. 32. DEFORMING LESIONS Advanced sarcoidosis with osteolytic lesions of the distal forearm, wrist, and bones of the hand<br />
  33. 33. SCLEROTIC LESION Rare and often in the axial skeleton.<br />
  34. 34. NASAL BONE LESION Nasal sarcoidosis can lead to osteolysis of the nasal bone (arrows).<br />
  35. 35. Heart<br />25% have cardiac involvement.<br />Sarcoidosis can affect any part of the heart.<br />Mostly Asymtomatic.<br />Screening should be done to rule out cardiac involvement in Sarcoidosis.<br />
  36. 36. Cardiac Sarcoidosis<br />Arrhythmias<br />Heart blocks- common<br />Heart failure- Restrictive to Dilated CMP.<br />Pericarditis- Effusions.<br />MI<br />SCD<br />
  37. 37. Cardiac Sarcoidosis<br />Difficult to Dx.<br />Screening and Dx can be done by :<br />ECG ,Holter monitoring ,ECHO<br />Nuclear imaging (with thallium and/or technetium sestamibi) <br />Cardiac PET/MRI <br />Heart biopsy, rarely /Never-can miss Dx –patchy involvement of myocardium.<br />
  38. 38. CNS<br />Neurosarcoidosis affects 15 percent of sarcoidosis patients. <br /> Some people with neurosarcoidosis will recover completely.  In others, sarcoidosis and related nervous system symptoms are chronic, lasting a long time or even a lifetime<br />
  39. 39. nerve inflammation and damage <br />peripheral neuropathy <br /><ul><li>Granulomas in the meninges (or more rarely in the brain) can lead to meningitis, hydrocephalus and neuroendocrine disorders . </li></li></ul><li>Cranial nerves, and peripheral nerves can be involved<br />7th nerve facial palsy is most common<br />Acute, transient, and can be unilateral or bilateral<br />HEREFORDT'S SYNDROME; facial palsy accompanied by fever, uveitis, and enlargement of the parotid gland <br />
  40. 40. MR IMAGE Temporal lobe sarcoid lesion (arrow)<br />
  41. 41. Nervous System<br />Optic nerve dysfunction-Blurring,doublevision,blindness.<br />seizures<br />Paresthesias<br />Encephalopathy <br />
  42. 42. <ul><li> CNS symptoms are not usually the first or only sign of sarcoidosis.</li></ul>Rarely Neurosarcoidosis is the only sign of sarcoidosis. <br />Isolated Neurosarcoidosis – Difficult to diagnose<br />CT/MRI/PET SCAN/LP will aid in Dx.<br />Biopsy rarely done.<br />
  43. 43. Kidney<br />Granulomatous interstitial nephritis produces renal failure<br />Develops over a period of weeks to months<br />Rapid response to steroid therapy<br />Kidney stones (nephrolithiasis) and nephrocalcinosis are very unusual secondary to hypercalcemia and hypercalciuria<br />
  44. 44. Kidney<br />Increased calcium absorption in the gut<br />Related to high levels of circulating 1,25-dihydroxy vitamin D produced by mononuclear phagocytes in granulomas<br />
  45. 45. Lymph Nodes<br />Lymphadenopathy<br />Intrathoracic nodes enlarged in 75-90% patients including hilar nodes and paratracheal nodes. <br />Peripheral lymphadenopathy<br />
  46. 46. Enlarged B/L Hilar, RT paratracheal .<br />
  47. 47. CALCIFIED LYMPH NODES late manifestation in 5% of patients.<br />
  48. 48. PARACARDIAC LYMPH NODE<br />
  49. 49. ABDOMINAL LYMPHADENOPATHY Multiple enlarged paraaortic, paracaval, and porta hepatis lymph nodes (arrows).<br />
  50. 50. GASTRIC SARCOID Granuloma involves the gastric antrum leading to irregular nonspecific narrowing.<br />
  51. 51. COLONIC SARCOID Irregular narrowing of the rectosigmoid has the appearance of inflammatory disease or malignancy.<br />
  52. 52. Lab Abnormalities<br />Lymphocytopenia<br />Mild eosinphilia<br />Increased E.S.R<br />Hyperglobulenemia<br />
  53. 53. Lab Abnormalities<br /><ul><li>ACE levels elevated 60- 80%.
  54. 54. Activated macrophages produce ACE.
  55. 55. Non Specific-Hodgkins,histoplasma,leprosy
  56. 56. ACE levels tested regularly to check the severity of the disease and to monitor the response to therapy.</li></li></ul><li>Radiography<br />CXR 3 classic patterns are seen.<br /> Type 1- bilateral hilaradenopathy with no parenchymal abnormalities.<br /> Type 2- bilateral hilaradenopathy with diffused parenchymal changes.<br /> Type 3- diffused parenchymal changes without hilaradenopathy. <br />
  57. 57. STAGE I Thoracic lymphadenopathy. Normal lung parenchyma. (50%)<br />
  58. 58. STAGE II Hilar and mediastinal lymphadenopathy. Abnormal lung parenchyma. ( 30% )<br />
  59. 59. STAGE III Abnormal lung parenchyma. No lymphadenopathy. ( 15% )<br />
  60. 60. STAGE IV Permanent lung fibrosis. (20%)<br />
  61. 61. MILIARY SARCOIDOSIS CT shows well defined lung nodules less than 5mm in diameter. This pattern is rare.<br />
  62. 62. ALVEOLAR SARCOIDOSIS Multiple lung masses are an unusual form of sarcoidosis, resembles lung metastases.<br />
  63. 63. Lung Function Test<br />Lung function abnormalities for interstitial lung disease with decreased lung volumes and diffusing capacities<br />
  64. 64. Radiography<br />“Egg shell” calcification of hilar nodes<br />Plural effusions<br />Cavitations<br />Atelectasis<br />Pneumothorax<br />Cardiomegaly<br />
  65. 65. Lymph nodes with rim (eggshell) calcification (arrow) are rare in sarcoidosis but common in silicosis.<br />
  66. 66. MOST COMMON PATTERN Bilateral symmetric hilar and right paratracheal mediastinal adenopathy.<br />
  67. 67. Diagnosis<br />Difficult to differentiate from chronic infections, fungal diseases, T.B. and lymphoma. <br />Based on combined clinical, radiologic and histologic findings. <br />Laboratory tests seldom important<br />
  68. 68. Diagnosis<br />Identify noncaseatinggranulomas<br />Transbronchial biopsies positive in 65-95%, even if no lung parenchymal abnormalities imaged. <br />Tissue from mediastinoscopy positive in 95%<br />Scalene node biopsy positive in 80%<br />
  69. 69. Diagnosis<br />KVEIM TEST-Like Mantoux. <br />Involves injecting standardized preparation of sarcoid tissue material into the skin.<br />Unique lump formed at the point of injection is considered positive for sarcoidosis. <br />
  70. 70. Diagnosis<br />Test not always positive <br />Test material not approved for sale by FDA. <br />
  71. 71. Differential Diagnosis<br />1.Hodgkin's disease:<br /> Mediastinal lymphadenopathy predominates in the anterior mediastinum and the paratracheal regions. <br />When lymphadenopathy involves the hilar regions, it is usually asymmetric.<br />
  72. 72. 2. Pulmonary tuberculosis:<br /><ul><li> Nodal enlargement is unilateral in about 80% of tuberculosis cases.
  73. 73. Lymph nodes are less well demarcated than in sarcoidosis.
  74. 74. Lymphadenopathy is almost always associated with ipsilateral lung disease</li></li></ul><li>3.Fungal infections:<br /><ul><li>Histoplasmosis
  75. 75. Coccidioidomycosis</li></ul> Lymphadenopathy may be unilateral or bilateral and is usually associated with parenchymal disease. Hilar and/or paratracheal lymph nodes may be involved.<br />
  76. 76. 4.Bronchogenic carcinoma:<br /> Unilateral hilar lymphadenopathy is common. <br />The appearance of the primary lung cancer (not visible in all cases) is often a large mass, which is unusual in sarcoidosis.<br />
  77. 77. 5.Metastatic lymph node enlargement: <br />Primary cancer in the kidney, prostate gland, or the UGI tract usually involves the middle mediastinal lymph nodes.<br />
  78. 78. Prognosis<br />Good<br />In 15-20% remains active or recurs intermittently. <br />50% have some permanent organ dysfunction<br />
  79. 79. Treatment<br />No known cure<br />Corticosteroids, primary treatment for inflammation and granuloma formation.<br />Prednisolone, 1 mg/kg for 4-6 weeks followed by slow taper over 2-3 months.<br />Abnormal cardiac/Neuro/Ocular /Hypercalcemia/Multi system – Steriods must.<br />
  80. 80. Treatment<br />CutaneousSarcoidosis- Topical steriods/Hydroxychloroquine.<br />Hydroxy chloroquine-200-400mg/day-Eye exam -6 monthly.<br />MTX- Preni not tolerated/not effective/steriod sparing agent.<br />MTX –Start with 10mg/week and maintain with 2.5 to 15mg/week.CBC,KFT,LFT/2months.<br />
  81. 81. Treatment<br /><ul><li>Infliximab-Monoclonal Ab to TNF- Improved lung function when given along with predni and MTX- recent RCT.
  82. 82. Rarely used.
  83. 83. Initial PPD –Reactivation of TB.</li></li></ul><li>THANK YOU.<br />

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