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
8. CLINICAL FEATURES
Asymptomatic Organ failure
Respiratory symptoms
Cutaneous manifestations
Ocular symptoms
Non specific constitutional symptoms
• Fatigue, ,fever, night sweats, LOW
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
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
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
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
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
