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

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  Systemic symptoms eg: 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 40- 90% 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