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Sarcoidosis<br />
Sarcoidosis<br /><ul><li>Sarcoidosis is a multisystem disorder  characterized by the presence of noncaseatinggranulomas.</...
Incidence and Prevalence<br /><ul><li>Disease can affect people of any age, race and gender.
20 to 40 years of age.
Most cases are Sporadic . 5 % of patients – FH of Sarcoidosis.</li></li></ul><li>Pathophysiology<br />Granulomas are Patho...
granulomas form in response to antigens that are resistant to "first-responder" inflammatory cells such as neutrophils and...
The antigenic triggering agents cause activation of the Helper T-cell and Macrophages <br /><ul><li>Activated Helper T-cel...
Giant cells are fused Macrophages-Langerhans giant cells. </li></ul>The central epithelioid and giant cells are surrounded...
All granulomas, regardless of cause, may contain additional cells and matrix. These include lymphocytes, neutrophils, eosi...
CYTOPLASMIC INCLUSION BODY Schaumann body (arrow) is common in sarcoidosis but is nonspecific.<br />
Clinical Manifestations<br />50% patients are asymptomatic<br />Abnormal "routine" chest radiograph<br />Symptomatic patie...
Clinical Manifestations<br />Respiratory symptoms are most common<br />Cough, chest discomfort, and dyspnea <br />Symptoms...
Lungs<br />First site involved<br />Begins with alveolitis involving small bronchi and small blood vessels <br />Alveoliti...
Noncaseating granuloma in lung is the characteristic lesion of sarcoidosis.<br />
CASEOUS NECROSISCellular destruction in TB granuloma appears as clumped debris (arrows). This necrosis does not occur in s...
M. tuberculosis BACILLI Caseous necrosis is most common in TB, but Gram negative, acid fast bacilli must be identified to ...
SUBPLEURAL GRANULOMA IN LUNG<br />
Eyes<br />25% have eye lesions<br />Blurred vision, pain, photophobia and dry eyes<br />Chronic uveitis leads to glaucoma,...
CONJUNCTIVITIS<br />
PAPILLEDEMA Often associated with 7th nerve facial palsy.<br />
Skin<br />33% have skin lesions<br />LOFGREN'S SYNDROME; acute triad of erythemanodosum, joint pains, and bilateral hilara...
NAKED GRANULOMA Young granulomas (arrows) in the skin with no surrounding rim of mononuclear cells.<br />
ERYTHEMA NODOSUM These reddish raised lesions. <br />
Skin<br />Lupus pernio- indurated blue purple swollen shiny lesions on nose, cheeks, lips, ears and fingers. <br />Papules...
LUPUS PERNIO <br />
RAISED PLAQUESThese raised plaques are the result of coalescence of nodules.<br />
PSORIASIS LIKE LESIONS These small white lesions closely resemble psoriasis.<br />
Liver<br />33% have hepatomegaly or biochemical evidence of disease <br />Symptoms usually absent<br />Cholestasis, fibros...
Musculoskeletal<br />Acute polyarthritis with fever is common<br />Arthritis is self limited<br />Chronic destructive bone...
PUNCHED OUT LYTIC LESIONS Focal osteolytic lesions in the fingers are most common abnormality.<br />
LACY TRABECULAR PATTERN Osteolysis has left a lacy trabecular pattern in this phalanx (arrow)<br />
DEFORMING LESIONS Advanced sarcoidosis with osteolytic lesions of the distal forearm, wrist, and bones of the hand<br />
SCLEROTIC LESION Rare and often in the axial skeleton.<br />
NASAL BONE LESION Nasal sarcoidosis can lead to osteolysis of the nasal bone (arrows).<br />
Heart<br />25% have cardiac involvement.<br />Sarcoidosis can affect any part of the heart.<br />Mostly Asymtomatic.<br />...
Cardiac Sarcoidosis<br />Arrhythmias<br />Heart blocks- common<br />Heart failure- Restrictive to Dilated CMP.<br />Perica...
Cardiac Sarcoidosis<br />Difficult to Dx.<br />Screening and Dx can be done by :<br />ECG ,Holter monitoring ,ECHO<br />Nu...
CNS<br />Neurosarcoidosis affects 15 percent of sarcoidosis patients. <br /> Some people with neurosarcoidosis will recove...
nerve inflammation and damage <br />peripheral neuropathy <br /><ul><li>Granulomas in the meninges (or more rarely in the ...
 MR IMAGE  Temporal lobe sarcoid lesion (arrow)<br />
Nervous System<br />Optic nerve dysfunction-Blurring,doublevision,blindness.<br />seizures<br />Paresthesias<br />Encephal...
<ul><li> CNS symptoms are not usually the first or only sign of sarcoidosis.</li></ul>Rarely Neurosarcoidosis is the only ...
Kidney<br />Granulomatous interstitial nephritis produces renal failure<br />Develops over a period of weeks to months<br ...
Kidney<br />Increased calcium absorption in the gut<br />Related to high levels of circulating 1,25-dihydroxy vitamin D pr...
Lymph Nodes<br />Lymphadenopathy<br />Intrathoracic nodes enlarged in 75-90% patients including hilar nodes and paratrache...
Enlarged B/L Hilar, RT paratracheal .<br />
CALCIFIED LYMPH NODES late manifestation in 5% of patients.<br />
PARACARDIAC LYMPH NODE<br />
ABDOMINAL LYMPHADENOPATHY Multiple enlarged paraaortic, paracaval, and porta hepatis lymph nodes (arrows).<br />
GASTRIC SARCOID Granuloma involves the gastric antrum leading to irregular nonspecific narrowing.<br />
COLONIC SARCOID Irregular narrowing of the rectosigmoid has the appearance of inflammatory disease or malignancy.<br />
Lab Abnormalities<br />Lymphocytopenia<br />Mild eosinphilia<br />Increased E.S.R<br />Hyperglobulenemia<br />
Lab Abnormalities<br /><ul><li>ACE levels elevated 60- 80%.
Activated macrophages produce ACE.
Non Specific-Hodgkins,histoplasma,leprosy
 ACE levels tested regularly to check the severity of the disease and to monitor the response to therapy.</li></li></ul><l...
STAGE I Thoracic lymphadenopathy. Normal lung parenchyma. (50%)<br />
STAGE II Hilar and mediastinal lymphadenopathy. Abnormal lung parenchyma. ( 30% )<br />
STAGE III Abnormal lung parenchyma. No lymphadenopathy. ( 15% )<br />
STAGE IV Permanent lung fibrosis. (20%)<br />
MILIARY SARCOIDOSIS CT shows well defined lung nodules less than 5mm in diameter. This pattern is rare.<br />
ALVEOLAR SARCOIDOSIS Multiple lung masses are an unusual form of sarcoidosis, resembles lung metastases.<br />
Lung Function Test<br />Lung function abnormalities for interstitial lung disease with decreased lung volumes and diffusin...
Radiography<br />“Egg shell” calcification of hilar nodes<br />Plural effusions<br />Cavitations<br />Atelectasis<br />Pne...
Lymph nodes with rim (eggshell) calcification (arrow) are rare in sarcoidosis but common in silicosis.<br />
MOST COMMON PATTERN Bilateral symmetric hilar and right paratracheal mediastinal adenopathy.<br />
Diagnosis<br />Difficult to differentiate from chronic infections, fungal diseases, T.B. and lymphoma. <br />Based on comb...
Diagnosis<br />Identify noncaseatinggranulomas<br />Transbronchial biopsies positive in 65-95%, even if no lung parenchyma...
Diagnosis<br />KVEIM TEST-Like Mantoux. <br />Involves injecting standardized preparation of sarcoid tissue material into ...
Diagnosis<br />Test not always positive <br />Test material not approved for sale by FDA. <br />
Differential Diagnosis<br />1.Hodgkin's disease:<br /> Mediastinal lymphadenopathy predominates in the anterior mediastinu...
   2. Pulmonary tuberculosis:<br /><ul><li> Nodal enlargement   is unilateral in about 80% of tuberculosis cases.
 Lymph nodes are less well demarcated than in sarcoidosis.
 Lymphadenopathy is almost always associated with ipsilateral lung disease</li></li></ul><li>3.Fungal infections:<br /><ul...
Coccidioidomycosis</li></ul>   Lymphadenopathy may be unilateral or bilateral and is usually associated with parenchymal d...
4.Bronchogenic carcinoma:<br /> Unilateral hilar lymphadenopathy is common. <br />The appearance of the primary lung cance...
5.Metastatic lymph node enlargement: <br />Primary cancer in the kidney,  prostate gland, or the UGI tract usually involve...
Prognosis<br />Good<br />In 15-20% remains active or recurs intermittently. <br />50% have some permanent organ dysfunctio...
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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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