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SARCOIDOSIS
Dr ABHINAND R S
DEPT:OF GENERAL MEDICINE
ESIC MC PGIMSR BANGALOE
Multisystem chronic inflammation characterized by
non caseating granulomas.
Multisystem chronic inflammation characterized by
non caseating granulomas.
1. BERYLLIOSIS
2. CROHN’S DISEASE
3. HSP(HYPERSENSITIVITY PNEUMONITIS)
4. CAT SCRATCH DISEASE
ETIOLOGY
• Disordered immune regulation in genetically predisposed individual
• Triggers may be infectious or non infectious environmental agent.
• Infectious agents-Propionibacter acnes
Mycobacterial protein (M.tuberculosis catalase
peroxidase [mKatG]
• Non infectious-Insecticides and mold
HLA SUSCEPTIBILITY
• HLA B8
• DR B1-03
• DR B1-04
• DR B1-02
• Women > Men
• 2/3 rd (50-70 %)-Spontaneous Resolution
• 20%- Chronic 5%-Death (mcc- ILD)
PATHOGENESIS
CLINICAL MANIFESTATIONS
• It can affect any organ system.
Asymptomatic(1/3) --------------------------Organ Failure
• Cough and dyspnea are the most common presenting symptoms.
• Other symptoms related to Ocular and Cutaneous.
• Nonspecific constitutional symptoms –Fatigue(mc),Fever,Night sweats
and weight loss .
SKIN
• B/L Hilar adenopathy
• Arthritis
• Erythema nodosum
• +Uveitis in 5% of patients
Lofgren’s Syndrome
• Uveitis
• Parotitis
• B/L LMN 7 th CN palsy
Heerfordt
waldenstorm
syndrome
Acute (Better Prognosis)
Extra—Causes for B/L LMN Facial Nerve palsy
• Guillain-Barre syndrome
• Sarcoidosis(Uveoparotid fever)
• Leprosy
• Diabetes mellitus
• Myasthenia gravis
• Lyme’s Disease
• Bulbar palsy
Chronic(Bad prognosis)
• Lupus Pernio
• Violaceous papule or Crusted erythematous maculopapular lesion on
face/nose dialated capillaries.
SKIN BIOPSY ASTEROID BODY
Stellate eosinophilic
EYES
• Chronic Anterior Uveitis( M C)
• Acute Anterior Uveitis
• Retinitis and parsplanitis
• Dry eyes (pre existing lacrimal gland
d’s)
ASYMPTOMATIC
BLINDNESS
LUNGS- m c organ affected(>90%)
Garland Sign
Lymphadenopathy of Rt & Lft
Hilar,RT paratracheal
staging on chest x ray (Scadding scoring system)
• stage I: hilar or mediastinal nodal enlargement only
• stage II: nodal enlargement and parenchymal disease
• stage III: parenchymal disease only
• stage IV: end-stage lung (pulmonary fibrosis)
Sarcoidosis involves upper lobe of lungs
others….
• Ankylosing spondylitis
• Psoriatic Arthritis
• Berylliosis
• Radiation Induced Lung Injury
• Hypersensitivity Pneumonitis
• Langerhans cell histiocytosis
• Silicosis
• TB
• Pneumocystis pneumonia
• ILD-NSIP
Restrictive pattern in spirometry –Reduced lung volumes
Decreased DLCO
• 50% pts –Obstructive pattern (reduced FeV1/FVC)
• 5% pts-Pulmonary Arterial Hypertension
CARDIAC
• Infiltration of the heart muscles by granuloma
CONGESTIVE HEART FAILURE
RESTRICTIVE CARDIOMYOPATHY> DIALATED CARDIOMYOPATY
CONDUCTION DEFECTS % HEART BLOCKS
ARRHYTHMIAS (multifocal) AND SUDDEN CARDIC DEATH
Diag:MRI / PET
Treat:Ablation not effective,ICD systemic therapy can be tried
HEPATIC
• INTRAHEPATIC CHOLESTASIS IS POSSIBLE-PORTAL HTN-OTHER
COMPLICATIONS.
RENAL
• CHRONIC TUBULOINTERSTITIAL DISEASE.
• M C GLOMARULAR DISEASE IS –MEMBRANEOUS NEPHROPATHY
METABOLIC
• Increased production of 1,25 dihydroxy vit D—increased absorption of
calcium from intestine---HYPERCACEMIA & REDUCED PTH LEVELS
BRAIN (5-10%)
• Cranial nerve involvement—B/l 7TH cranial nerve palsy & Optic neuritis.
• Pituitary stalk & anterior hypothalamus involvement—CENTRAL D I
• Transverse myelitis
• Sizures and cognitive changes.
MRI with g-space occupying lesions,CSF-lymphocytic meningitis with mildly
elevated protein.
DIAGNOSIS
• OLD METHOD- Ga 67 scan
• NEWER MODALITY—PET SCAN & BIOPSY
• ACE LEVELS NORMAL CAN RULE OUT SARCOIDOSIS
HIGH ACE LEVELS
• Gaucher’s disease
• Miliary TB
• Leprosy(HD)
• Hyperthyroidism
• BAL-CD4/CD8—HIGH(> 3.5 times strongly supportive)
• Kviem –Siltzbach procedure—specific diagnostic test for sarcoidosis
I/D injection sarcoid pt’s spleen preparation---Biopsied after 4-6
weeks of injection—non caseating granuloma confirms diag:
Treatment
• Acute—organ system inv—systemic steroids
• Chronic—Steroids+mtx/azathioprine/chloroquine
second line:antiTNF alfa -Infliximab

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Sarcoidosis

  • 1. SARCOIDOSIS Dr ABHINAND R S DEPT:OF GENERAL MEDICINE ESIC MC PGIMSR BANGALOE
  • 2. Multisystem chronic inflammation characterized by non caseating granulomas.
  • 3. Multisystem chronic inflammation characterized by non caseating granulomas. 1. BERYLLIOSIS 2. CROHN’S DISEASE 3. HSP(HYPERSENSITIVITY PNEUMONITIS) 4. CAT SCRATCH DISEASE
  • 4. ETIOLOGY • Disordered immune regulation in genetically predisposed individual • Triggers may be infectious or non infectious environmental agent. • Infectious agents-Propionibacter acnes Mycobacterial protein (M.tuberculosis catalase peroxidase [mKatG] • Non infectious-Insecticides and mold
  • 5. HLA SUSCEPTIBILITY • HLA B8 • DR B1-03 • DR B1-04 • DR B1-02
  • 6. • Women > Men • 2/3 rd (50-70 %)-Spontaneous Resolution • 20%- Chronic 5%-Death (mcc- ILD)
  • 8. CLINICAL MANIFESTATIONS • It can affect any organ system. Asymptomatic(1/3) --------------------------Organ Failure • Cough and dyspnea are the most common presenting symptoms. • Other symptoms related to Ocular and Cutaneous. • Nonspecific constitutional symptoms –Fatigue(mc),Fever,Night sweats and weight loss .
  • 9. SKIN • B/L Hilar adenopathy • Arthritis • Erythema nodosum • +Uveitis in 5% of patients Lofgren’s Syndrome • Uveitis • Parotitis • B/L LMN 7 th CN palsy Heerfordt waldenstorm syndrome Acute (Better Prognosis)
  • 10. Extra—Causes for B/L LMN Facial Nerve palsy • Guillain-Barre syndrome • Sarcoidosis(Uveoparotid fever) • Leprosy • Diabetes mellitus • Myasthenia gravis • Lyme’s Disease • Bulbar palsy
  • 11. Chronic(Bad prognosis) • Lupus Pernio • Violaceous papule or Crusted erythematous maculopapular lesion on face/nose dialated capillaries.
  • 12. SKIN BIOPSY ASTEROID BODY Stellate eosinophilic
  • 13. EYES • Chronic Anterior Uveitis( M C) • Acute Anterior Uveitis • Retinitis and parsplanitis • Dry eyes (pre existing lacrimal gland d’s) ASYMPTOMATIC BLINDNESS
  • 14. LUNGS- m c organ affected(>90%)
  • 15. Garland Sign Lymphadenopathy of Rt & Lft Hilar,RT paratracheal
  • 16. staging on chest x ray (Scadding scoring system) • stage I: hilar or mediastinal nodal enlargement only • stage II: nodal enlargement and parenchymal disease • stage III: parenchymal disease only • stage IV: end-stage lung (pulmonary fibrosis)
  • 17. Sarcoidosis involves upper lobe of lungs others…. • Ankylosing spondylitis • Psoriatic Arthritis • Berylliosis • Radiation Induced Lung Injury • Hypersensitivity Pneumonitis • Langerhans cell histiocytosis • Silicosis • TB • Pneumocystis pneumonia
  • 18. • ILD-NSIP Restrictive pattern in spirometry –Reduced lung volumes Decreased DLCO • 50% pts –Obstructive pattern (reduced FeV1/FVC) • 5% pts-Pulmonary Arterial Hypertension
  • 19. CARDIAC • Infiltration of the heart muscles by granuloma CONGESTIVE HEART FAILURE RESTRICTIVE CARDIOMYOPATHY> DIALATED CARDIOMYOPATY CONDUCTION DEFECTS % HEART BLOCKS ARRHYTHMIAS (multifocal) AND SUDDEN CARDIC DEATH Diag:MRI / PET Treat:Ablation not effective,ICD systemic therapy can be tried
  • 20. HEPATIC • INTRAHEPATIC CHOLESTASIS IS POSSIBLE-PORTAL HTN-OTHER COMPLICATIONS. RENAL • CHRONIC TUBULOINTERSTITIAL DISEASE. • M C GLOMARULAR DISEASE IS –MEMBRANEOUS NEPHROPATHY
  • 21. METABOLIC • Increased production of 1,25 dihydroxy vit D—increased absorption of calcium from intestine---HYPERCACEMIA & REDUCED PTH LEVELS BRAIN (5-10%) • Cranial nerve involvement—B/l 7TH cranial nerve palsy & Optic neuritis. • Pituitary stalk & anterior hypothalamus involvement—CENTRAL D I • Transverse myelitis • Sizures and cognitive changes. MRI with g-space occupying lesions,CSF-lymphocytic meningitis with mildly elevated protein.
  • 23. • NEWER MODALITY—PET SCAN & BIOPSY • ACE LEVELS NORMAL CAN RULE OUT SARCOIDOSIS HIGH ACE LEVELS • Gaucher’s disease • Miliary TB • Leprosy(HD) • Hyperthyroidism
  • 24. • BAL-CD4/CD8—HIGH(> 3.5 times strongly supportive) • Kviem –Siltzbach procedure—specific diagnostic test for sarcoidosis I/D injection sarcoid pt’s spleen preparation---Biopsied after 4-6 weeks of injection—non caseating granuloma confirms diag:
  • 25. Treatment • Acute—organ system inv—systemic steroids • Chronic—Steroids+mtx/azathioprine/chloroquine second line:antiTNF alfa -Infliximab