Clinical Features of Sarcoidosis and Basic Treatment Options for Final Year Medical Students

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