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Sarcoidosis
- 1. SARCOIDOSIS: CLINICAL FEATURES DIAGNOSIS & TREATMENT DR ASWIN RM
- 3. SARCOIDOSIS Multi system C/c inflammatory condition Non caseating granulomas Either resolves or proceeds to fibrosis
- 4. HISTORY • First described in by Dr. Jonathan Hutchinson1877 • Term Lupus pernio was coined - Dr. Ernest Besnier1888 • Histology defined by Norwegian dermatologist Boeck1899 • Bone involvement first described1902 • Uveitis in sarcoidosis first described1910 • Systemic condition , by Dr. Schaumann1915 • Lung involvement1915 • Uveoparotid fever1937 • Löfgren syndrome1941 BOECK BENSIER SCHAUMANN DISEASE
- 5. ETIOLOGY Exact aetiology Uncertain Environmental factors • Infective • Non infective Genetic factors • HLA-DRB1*1101 • Other genes
- 6. PATHOGENESIS SARCOID GRANULOMA Non Caseating Naked granuloma Inclusion bodies • Schaumann bodies • Asteroid bodies • Reesidual bodies • Crystalline inclusions Rarely Necrosis + Can calcify
- 7. EPIDEMIOLOGY • F>M • 3rd decade • Almost all races
- 8. CLINICAL FEATURES Asymptomatic Organ failure Respiratory symptoms Cutaneous manifestations Ocular symptoms Non specific constitutional symptoms • Fatigue, ,fever, night sweats, LOW
- 10. LUNGS ~95 % 50%- permanent lesions 5- 15 % Progressive fibrosis of lung Lymph node infiltration Interstitial infiltration Airway disease Pleural effusion
- 11. LUNGS Cough DOE Chest pain Obstructive symptoms Haemoptysis rarely Asymptomatic
- 12. CHEST XRAY Scoring system -By Scalding -1961 -4 stages NODULAR SARCOID • multiple, bilateral lung nodules • minimal hilar adenopathy CONSOLIDATION MILIARY OPACITIES LINEAR RETICULAR OPACITIES CAVITIES Stage 1 BHL Stage 2 BHL + infiltrates Stage 3 infiltrates alone Stage 4 Fibrosis
- 13. CT CHEST Hilar and mediastinal lymphadenopathy Peri Bronchial wall thickening Reticulonodular opacities Thickening of the bronchovascular bundles Nodules along bronchi, vessels, and subpleural regions Traction bronchiectasis Fibrosis with distortion of the lung architecture , cavities
- 14. S • Sarcoidosis • Silicosis H • Hyper sensitivity pneumonitis • Langerhans cell histiocytosis A • Ankylosis spondilitis R • Radiation Pneumonitis P • PCP • PTB UPPER LOBE INFILTRATES -SHARP
- 15. B/L HILAR ADENOPATHY Infection Mycobacteria (G) Fungal (G) Mycoplasma HIV Malignancy Lymphoma (G) Lymphamatoid granulomatosis (G) Lung cancer Metastasis Inorganic dust Silicosis (G) Berylliosis (G) ANCA associated (rare) Wegeners granulomatosis (G) Churg strauss (G) Others Hypersensitivity pneumonitis (G) Langerhan cell Histiocytosis (G) Lymphocytic interstitial pneumonia (G)
- 16. AIRWAY DISEASE • 50% has some obstructive disease • Larger or smaller airways • Many responds to bronchodilators and steroids PLEURAL DISEASE • Rare <1% • Pleural effusion • Chylothorax
- 17. OTHER FINDINGS PFT • Decreased Lung volume • 50% obstructive findings DLCO • Reduced PAH • 5% • Direct vascular involvement • Fibrosis
- 18. SKIN Maculo papular Lupus pernio Eryhtema nodosum Subcutaneous nodules Hypopigmented Nodular sarcoidosis 1/3rd PATIENTS SPECIFIC AND NONSPECIFIC
- 19. SKIN Plaque Ulcerative Verrucous Angiolipoid Scar sarcoidosis
- 20. Erythema Nodosum Polyarthritis Fever Hilar adenopathy LÖFGREN SYNDROME • Good prognosis
- 21. DIAGNOSIS • Skin biopsy – Non caseating granulomas DIFFERENTIAL DIAGNOSIS • Foreign body reaction • Lupus vulgaris • Tuberculoid leprosy • Drug eruptions • DLE
- 22. EYE Most common – anterior uveitis Intermediate uveitis 1/3rd chorioretinits Retinal vasculitis Keratoconjunctivitis Asymptomatic
- 23. OTHER OCULAR MANIFESATIONS • Secondary glaucoma • Cataract formation • Blindness LATE COMPLICATIONS IN UNTREATED PATIENTS • Presents as palpable orbital mass • Lacrimal gland • Extraocular muscles • Optic sheath EXTRA OCULAR MANIFESTATIONS
- 24. HEERFORDT SYNDROME Fever Uveitis Parotid Enlargement Facial palsy HEERFORDT SYNDROME
- 25. LIVER • Granulomatous lesions in 50%Liver biopsy • Abnormal in 20-30% • Elevated ALP -MC abnormality • Elevated transaminases LFT • Majority asymptomatic • Due to intrahepatic cholestasis & PHTN • Due to hepatomegaly Symptoms • Liver transplant rarely – good response to systemic Rx • Never give interferon Rx to co existant Hepatitis COther features
- 26. NERVOUS SYSTEM Cranial N palsies Optic Neuropathy Peripheral Neuropathies Granulomatous lesions in brain and spinal cord • Parenchymal nodules • Neuroendocrine manifestations • Seizures • Cognitive dysfunction • FND • Meningitis • Spinal cord syndromes Vascular and perivascular involvement
- 27. NERVOUS SYSTEM FINDINGS CSF • Lymphocytic pleocytosis • Elevated protein • Elevated ACE IMAGING • Contrast enhanced MRI BIOPSY
- 28. SPLEEN ,BONE MARROW & LYMPH NODES Splenic Granulomas ~ 60 % Splenic enlargement 5-10 % Features of hypersplenism Rarely splenectomy needed • Massive symptomatic splenomegaly • Pancytopenia MC haematological abnormality – lymphopenia • Due to sequestration in areas of inflammation Anemia ~ 20% Extrathoracic L/N ~ 20%
- 29. HEART HEART FAILURE • Diffuse granulomatous involvement of myocardium • Improves with Rx VALVULAR DISEASE CONDUCTION BLOCK • CHB Most common cardiac abnormality TACHYARHYTHMIAS & SCD • Common cause of death • due to patchy involvemnt, ablation therapyis not useful. • implanted defibrillator may be needed
- 30. DIAGNOSIS ECG • Routine ECG & Holter monitoring ECHOCARDIOGRAM CARDIAC MRI or PET SACN ENDOMYOCARDIAL BIOPSY
- 31. MUSCULOSKELETAL 5% ACUTE ARTHRITIS • Isolated or as part of Lofgren syndrome CHRONIC ARTHRITIS • Oligo or polyarticular • Joint erosions and periosteal bone resorption SARCOID MYOPATHY • Infiltration of muscle MYALGIAS AND ARTHRALGIAS – MC COMPLAINT XRAY, MRI , PET or ISOTOPE SCAN , muscle biopsy , EMG
- 32. OTHER ORGANS • Direct involvement – 5 % • More symptomatic renal d/s secondary to hypercalcemia KIDNEY • Hypercalcemia and/or hypercalciuria • 1,25-dihydroxyvitamin D by the granuloma Ca METABOLISM UPPER RESPIRATORY TRACT • Diffuse goiter or, rarely solitary thyroid nodule • All patients are but euthyroid THYROID • Breast, testes, ovary & stomach.USUALLY SPARES
- 33. OTHER INVESTIGATIONS Kviem- Siltzbach Test GALLIUM SCAN PET MRI ACE CD4/CD8 BAL/viteous
- 34. DIAGNOSIS a compatible clinical and/or radiological picture histological evidence of non caseating granulomas exclusion of other diseases with similar histological or clinical picture.
- 35. DIAGNOSIS Biopsy showing granuloma No alternative diagnosis Clinically consistent with sarcoidosis Yes SARCOIDOSIS No Possible sarcoidosis Seek other DD Features suggesting Sarcoidosis CXR , Skin lesions, uveitis, optic neuritis, hypocalcemia, hypercalciuria, Cr N palsies Biopsy affected organ if possible Bronchoscopy: biopsy with granuloma Needle aspirate: granulomas Negative but no evidence of alternative diagnosis Features highly consistent with sarcoidosis: Serum ACE > 2 times UL BAL lymphocytes > 2 times UL Positive Gallium scan No Yes Positive and no alternative diagnosis SARCOIDOSIS
- 36. TREATMENT Abnormal LFT , Abnormal CXR - monitoring Heart Eye Nervous system
- 37. TREATMENT Acute illness Minimal to No organs Abnormalities of neurologic cardiac, ocular, calcium YES – Systemic therapy NO- no therapy and observe Single Organ Disease Only anterior eye , localised skin , cough YES- Topical therapy NO- systemic therapy Symptomatic Multiple Organs Systemic therapy with glucocorticoids Taper to <10 mg in <6 months: continue prednisone Cannot taper to <10g or Steroid toxicity Consider Mtx , HCQ, Azathioprine
- 38. TREATMENT Chronic illness Glucocorticoids tolerated Dose <10 mg/d YES – Continue therapy NO- Seek alternative agents Glucocorticoids not tolerated Alternative agents Methotrexate HCQ Azathioprine Mycophenolate Minocycline Glucocorticoids not effective Try alternative agents If effective, taper off glucocorticoids If not effective, consider: Multiple agents Infliximab Cyclophosphamide Thalidomide
- 39. Non Steroidal agents • Chloroquine – Cutaneous lesions , hypercalcemia ,bone lesions • Minocycline – cutaneous lesions • Methotrexate • Azathiprine • Mycophenolate • Cyclophosaphamide • Thalidomide • Infliximab
- 40. INDICATIONS IN PULMOARY SARCOID Bothersome or worsening symptoms • cough, shortness of breath, chest pain or discomfort, hemoptysis). Deteriorating lung function, • serial testing at three to six month intervals, that demonstrates one or more of the following: • a fall in total lung capacity (TLC) of 10 percent or more; • a fall in forced vital capacity (FVC) of 15 percent or more; • a decrease in diffusing capacity (DLCO) of 20 percent or more; • or worsened gas exchange at rest (eg, a decrease in oxygen saturation on pulse oximetry of four or more percent) Severe enough initial disease • (FEV 1 ) below 70 percent of predicted, • a diffusing capacity below 60 percent of predicted, and/or • an oxygen saturation of 90 percent or less, and • widespread radiographic opacities. Progressive radiographic changes • worsening of interstitial opacities, • development of cavities, • progression of fibrosis with honeycombing, or • development of signs of pulmonary hypertension.
- 41. ADVERSE PROGNOSTIC FACTORS • Lupus pernio • Chronic uveitis • Age of onset > 40 yrs • Chronic hypercalcaemia • Nephrocalcinosis • Black race • Nasal mucosal involvement • Cystic bone lesions • Neurosarcoidosis • Myocardial involvement
- 42. THANK YOU