Sarcoidosis is a multisystem granulomatous disease of unknown etiology characterized by the formation of non-caseating granulomas. It commonly involves the lungs and can cause pulmonary symptoms as well as extrapulmonary manifestations affecting the skin, eyes, heart and lymph nodes. Diagnosis is made through biopsy demonstrating non-caseating granulomas and treatment involves NSAIDs, corticosteroids, and steroid-sparing immunosuppressants depending on symptoms and organ involvement.

1. Sarcoidosis
Presented By:
Mohamad Kayali
Mariam Abdulrahman

2. What is Sarcoidosis
 A GRUELLING Disease
 ACE increase
 Granulomas
 ARthritis
 Uveitis
 Erythema Nodosum
 Lymphadenopathy (B/L Hilar)
 Lupus Pernio
 Idiopathic
 Noncaseating
 Gammaglobinemia
 Vit D increase (Hypercalcemia)

3. Definition
 Unknown Etiology
 Multisystem
 Lungs
 Eyes
 Skin
 Heart
 Joints
 Granulomatous
 Noncaseating
 Epidemiology:
 African
 Females
 Young

4. Presentation
 Asymptomatic
 Incidental on CXR
 Symptomatic
 Pulmonary Symptoms
 Constitutional Symptoms
 Extra-pulmonary Symptoms
50%
50%
30%

5. Pulmonary Manifestations
 Symptoms:
 Cough
 SOB
 Wheeze
 Vague chest pain or discomfort
 Physical examination is unremarkable
 Rarely fine inspiratory crackles (Pulmonary Fibrosis)
 B/L Hilar Lymphadenopathy

6. Constitutional Symptoms
 Fatigue
 Fever
 Malaise
 Weight loss
 Night sweats

7. Extrapulmonary
Manifestation
 Cardiac Manifestations:
 Arrhythmias: Commonly Complete Heart Block
 Heart Failure: Cor-Pulmonale
 Skin:
 Specific Eruptions;
 Lupus Pernio
 Scar and Tattoo Sarcoids
 Nonspecific Eruptions;
 Erythema Nodosum
 Eyes:
 Uveitis
 Other Structures: Visual blurring

8. Extrapulmonary
Manifestations
 Lymph nodes, Liver and Spleen
 Lymphadenopathy (B/L Hilar; 90%)
 Hepatosplenomegaly (less common)
 Neurological
 Commonly Mononeuropathy of CN 7 (50%)
 Exocrine;
 Painless swelling of parotid gland
 Renal:
 Hypercalciuria; Nephrolithiasis (increased activation of Vit D by
macrophages)
 MSK:
 Acute polyarthritis

9. Syndromes Related to
Sarcoidosis
 Lofgren Syndrome:
 Triad of:
 Hilar Lymphadenopathy
 Erythema Nodosum
 Polyarthritis
 Resolve Spontaneously (NSAIDs to control symptoms)
 Mikulicz’s Syndrome:
 Salivary and Lacrimal gland

10. Differential Diagnosis
 TB
 Caseating Granulomas
 Lymphoma
 Vasculitis
 Granulomatosis with Polyangiitis (Wegner’s)
 Pulmonary symptoms (Cough, Dyspnea,
Hemoptysis) and granulomas (Necrotizing)
 c-ANCA (PR-3)
 Churg Strauss
 A/W Asthma and granulomas (Necrotizing)
 p-ANCA (MPO)

11. Differential Diagnosis
 Immune
 Langerhans Cells Histiocytosis (Bone Lesions, Skin)
 Common Variable Immune Deficiency (Low IGs, Lymphomas, Pulmonary
Infections)
 Infections
 Fungal Infections: Histoplasmosis (Caseating Granulomas)
 Bacterial:
 Bartonella henselae (Cat Scratch, Noncaseating granuloma with
stellate necrosis)
 Brucellosis (unpasteurized dairy)
 Chlamydia (LGV)
 Exposure
 Berylliosis
 Noncaseating granulomas in lungs
 Aerospace industry

12. Diagnosis
 Initially: Chest X Ray
 B/L Hilar Lymphadenopathy
 Diagnostic: Biopsy revealing non-caseating
granuloma
 Lung tissue: Bronchoscopy and Transbronchial Lung
Biopsy
 Lymph nodes:
 Endobronchial Ultrasound with Transbronchial FNA of
Lymph nodes
 Mediastinoscopy/VATS
 Skin Biopsy
 PPD Skin Test (Mantoux)

13. Other Investigations
 Bronchoalveolar Lavage
 Elevated CD4+/CD8+
 Pulmonary Function Tests
 Restrictive Pattern
 FBC & Biochemistry:
 U/E: Hypercalcemia
 LFTs and RFTs
 Urine:
 Urinalysis (Stones)
 24 hr Urinary Calcium (Hypercalciuria)
 Serum ACE
 Non specific
 Not a clear role in monitoring response to treatment
 EKG
 Conduction Abnormalities
 Eye Examination

14. Radiographic Staging
 Based on CXR
 Stage 1: B/L Hilar LAN
 Stage 2: B/L Hilar LAN + Infiltrates (Reticular
Opacities)
 Stage 3: Infiltrates only
 Stage 4: Signs of Fibrosis

15. Radiographic Staging
Stage 1 Stage 2
Stage 3 Stage 4

16. Screening for complications
HRCT Thorax (pulmonary fibrosis)
ABG (hypoxia/respiratory failure)
ECG/Holter (cardiac arryythmia)
Echo (pulmonary hypertension)
Right heart catheterization (pulmonary
hypertension)
24 hour urinary calcium (hypercalciuria)
CT Brain (hydrocephalus)

17. Acute Management
 NSAIDs
 Topical or oral
 Oral corticosteroids
 Prednisolone 20-40 mg od po and taper

18. Long term management
Monitoring
Asymptomatic
Watch and
wait approach
Symptomatic
Treat
symptoms
Clinical exam
PFT
CXR
Serum ACE

19. Indications for systemic
treatment
 Progressive dyspnoea
 Worsening pulmonary infiltrates
 Fall in FVC and/or DLCO
 Anterior or posterior uveitis unresponsive to topical
therapy
 Active cardiac involvement
 Hypercalcaemia
 CNS involvement (Facial Nerve/ CN VII in 50%)

20. Long term Treatment
• If
available
•Anti-TNF alpha:
infliximab,
pentoxyfylline,
thalidomide
•Methotrexate,
Azathioprine,
Hydroxychloroq
uine
•Topical or
Systemic
•+Add PPI
/bone
prophylaxis
Steroids
Steroid
sparing
agents
Lung
transplant
Immune-
Modulato
rs

21. Side effects of Steroids?
 Topical vs systemic
 Iatrogenic Cushing
 Acne
 Diabetes Mellitus
 Thinning of skin
 Osteoporosis

22. Complications
 Pulmonary fibrosis
 Pulmonary hypertension
 Respiratory failure
 Lung transplantation
 Cardiac arrhythmia, sudden cardiac death 20 to ventricular
fibrillation
 Hypercalcaemia
 Chronic renal failure 20 to Renal sarcoid
 Glaucoma and visual loss
 Obstructive hydrocephalus 20 to Neurosarcoid

23. Prognosis
 Spontaneous resolution of symptoms in 1/3
 60-80% do not require treatment
 Progressive lung disease in up to 20%
 1-5% of patients die of a complication of
sarcoidosis

24. Adverse prognostic factors
include
 Age of onset > 40 years
 African ethnicity
 Pulmonary sarcoidosis
 Pulmonary hypertension
 Cardiac and neurological involvement
 Chronic hypercalcaemia
 Lupus pernio
 Chronic uveitis
 Nephrocalcinosis
 Nasal mucosal involvement

25. Summary
 Sarcoidosis is a multi-system diffuse lung parenchymal
disease leading to scaring (fibrosis).
 Pulmonary, skin and constitutional symptoms
 Look out for B/L Hilar Lymphadenopathy on CXR
 Biopsy is diagnostic revealing non-caseating granuloma
 Treatment includes NSAID and Steroids.
 Long term Treatment, Steroids, Steroid sparing, Anti TNF alpha
and Lung transplant