1. Sarcoidosis

2. SARCOIDOSIS
Definition: Idiopathic systemic disorder characterized by accumulation of lymphocytes and monocytes in many organs forming noncaseating, epitheloid granuloma and subsequent conformational changes in the involved organs
Etiology: unknown
Extent of involvement : systemic
Clinical course : variable from asymptomatic disease with spontaneous resolution to progressive disease with organ system failure
Symptoms: dependent on site of involvement 1

3. Typically affects young adults between 20-60 years of age
Most frequently affects the lungs but some can present with extrathoracic manisfestation.
Diffuse interstitial lung disease is the classic type of lung involvement
Common presenting respiratory symptoms includes:
Cough
Dyspnoea
Chest pain
Also can present with systemic symptoms like fatigue, anorexia and skin lesion

4. Diagnosis: Chest radiograph
Bilateral hilar adenopathy is a classic finding in sarcoidosis
Hilar may be symmetrically enlarged or the right may be slightly more prominent
CXR findings are organized into stages (I-IV) which gives an anatomic guide to lung involvement

5. Diagnosis: Chest radiograph
Stage I
Presence of bilateral hilar adenopathy which is often accompanied by paratracheal node enlargement.
Regression of hilar nodes within 1-3 years occur in 75% of patients
Stage II
Consist of bilateral hilar adenopathy and reticular opacities (upper lung zone more than lower)
Usually have mild to moderate symptoms (cough, dyspnea, fever and fatigue)

6. Stage I
Stage II

7. Diagnosis: Chest radiograph
Stage III
Consist of reticular opacities with shrinking hilar nodes
Predominantly distributed in upper zones
Stage IV
Reticular opacities with evidence of volume loss
Marked traction bronchiectasis, extensive calcification and cavitation may also be seen

8. Stage III
Stage IV

9. Diagnosis: Chest radiograph
HRCT can detect parenchymal and mediastinal abnormalities not seen on CXR. Eg:
Hilar and mediastinal lymphadenopathy
Beaded or irregular thickening of bronchovascular bundles
Nodules along bronchi, vessels and subpleural regions
Bronchial wall thickening
Ground glass opacification
Parenchymal masses or nodular consolidation, occasionally with cavitation
Fibrosis with distortion of the lung architecture and traction bronchiectasis

10. Pulmonary Function Test
PFTs & 6MWT are used to assess the severity of respiratory impairment and monitor course of disease
PFTs usually shows a restrictive pattern with a reduction in DLCO
Serum ACE
Activity elevated in % due to macrophage activity, but nonspecific
Lacks diagnostic specificity and poor prognostic value in identifying patients with progressive disease

11. Bronchoscopy
Bronchoscopy with BAL, endobronchial biopsy and transbronchial biopsy are tradiotnal methods for minimally invasive diagnosis of sarcoidosis
BAL demonstrates:
a reduced number of CD8 cells
elevated CD4 to CD8 ratio
increased amount of activated T cells, CD4 cells and immunoglobulins
EUS or EBUS has a high diagnostic yield in patients with mediastinal adenopathy and a clinical suspicion of sarcoidosis

12. Kveim test Kveim test, Nickerson-Kveim or Kveim-Siltzbach test
Splenic cells from patients with sarcoidosis is injected intradermally to evoke a sarcoid granulomatous response over 3 weeks.
Not commonly performed due to concerns on disease transmission

13. Extrapulmonary features
Periperal lymph node involvement
Myocardial involvement 5-10%
arrhythmias, heart failure (restrictive type), conduction abnormalities
Occular involvement
Anterior/posterior uveitis
Conjunctivitis
Skin
plaques, papules, subcutaneous nodules
keloid formation in atrophic scars
erythema nodosum
Lupus pernio - violaceous, chronic and disfiguring lesions of the ears, nose and cheeks

14. Extrapulmonary features
Neurological disease – facial palsy
Other organs eg:
Liver
Joints
Splenomegaly
Lacrimal gland
Parotid gland
Bone involvement
Sarcoid dactylitis

15. Lupus Pernio

16. TREATMENT Corticosteroids Cytotoxic agents:
methotrexate, azathioprine, chlorambucil,
cyclophosphamide
Other agents : antimalarials, ketoconazole,
NSAID’s
• Infliximab

17. Treatment
Treatment of sarcoidosis is usually limited to symptomatic patients
Treatment decision have to consider that patients may have spontaneous resolution of the disease
Criteria for institution of glucocorticoid therapy
Disabling symptoms (Fever, Arhtralgias, Cough, Dyspnoea, Chest discomfort, Exercise limitation)
Organ dysfunction/derangement
Abnormal tests (Hypercalcaemia, Abnormal LFTs)
Ancillary criteria (Elevated levels of BAL lymphocytes