SARCOIDOSIS

D R. C O L . VI S H A L M A R W A H A
MD, DNB, MNAMS
 INTRODUCTION

• Multisystem granulomatous disorder

• Lungs affected in >90% cases

• Characteristic non-caseating granulomas in tissues

• Acute, Subacute or self limiting course.

• Chronic ‘WAXING & WANING’ course also seen.

 Some Important Terms

* 1909 Heerfordt discussed Uveoparotid fever

comprising Uveitis, enlarged salivary glands and
facial palsy

* 1942 – Lofgren described Lofgren syndrome

comprising erythema nodosum, bilateral hilar
lymphadenopathy and febrile arthropathy
eg:- Ankle joint.
 EPIDEMIOLOGY

• Both sexes
• All ages, races and geographical areas
• Female more likely to have eye lesions, neurologic involvement and erythema
nodosum
• Male more likely to have hypercalcemia
• Blacks – Chronic skin lesions, eye, liver, bone marrow and lymph node involvement
• More common in US- African Americans (10:1 to 17:1) while whites are more
affected in Europe.
• Most patients – age group is 20 – 40 years
• Favours – Non smokers
• India  Confusion with TB but now more cases are reported

ETIOLOGY – Not clear

Propionibacterium granulosum DNA found in patients in Europe and Japan
 CLINICAL FEATURES

1. Abnormal Chest X ray

2.Severe Multi organ involvement
3.Chronic dry cough
4.Chronic dyspnea- Rare – associated with advanced pulmonary fibrosis
5. Peripheral lymph nodes
6.Eyes- Uveitis
7. Skin – Erythema Nodosum
8.20 – 40 % present with acute or subacute SARCOIDOSIS of few weeks
duration with constitutional symptoms. Some present with cough,
dyspnea, retrosternal discomfort and/or polyarthritis
9.Insidious form (40-70%) develops over months and usually present
with respiratory symptoms
 Intra-Thoracic Manifestations

Pulmonary involvement – 95%

a) Asymptomatic hilar lymphadenopathy (50-60%)
b) ILD with alveolitis
c) Pleural effusion are rare (<5%)
d) Endobronchial involvement found in biopsy in 50% and may lead to
airway stenosis (10%)
e) Symptoms – i) Dry Cough (30%)
ii) Dyspnea (28%)
iii) Chest pain (15%)
f) Wheezing – endobronchial involvement
g) Hemoptysis – rare although can occur in patients with fibrosis and
cavities filled with aspergillomas
h) Lung Crackles – only in 20%
i) Clubbing is rare
 Stage I(Lymphadenopathy) Stage II (Lymphadenopathy & Infiltrates)
STAGES OF SARCOIDOSIS – I to IV

Stage III (Infiltrates only) Stage IV (Fibrosis)

 Ocular Manifestations

1. 25 %  5% as initial presentation
2. Acute anterior bilateral granulomatous uveitis is common manifestation
3. Baseline eye exam (SLIT exam) – is very important as 1/3rd develop
asymptomatic eye involvement
4. Other findings may occur
a) Interstitial Keratitis
b) Posterior Uveitis
c) Pars Planitis ( Snowballing in slit examination)
d) Scleral Plaques
e) Lacrimal gland enlargement (15-28 %)
f) Optic neuropathy
g) Corneal/ conjunctival nodules
Granulomatous Anterior Uveitis
 SKIN/Lupus Pernio

1. 30 % cases
2. Sarcoid specific lesion  Granuloma seen on biopsy
Hyperpigmented maculopapular lesion ( 2-5 mm ) seen on
face, nape of neck and upper back
3. Other specific lesion – i) Skin Plaques
ii) Annular lesion ( Poor prognosis )
4. Lupus Pernio – Charactersistic skin lesion. It is associated
with chronic progressive upper and lower airway disease
and bony involvement
5. Non specific lesion – Erythema Nodosum – inflammatory
( no granulomas )
Lupus Pernio - Image
 Cardiac Manifestations

1. Conduction disturbances
2. Papillary muscle dysfunction
3. Infiltrative Cardiomyopathy with CHF
4. Cor Pulmonale due to severe restrictive lung disease and pericarditis
5. Investigations
i) Holter
ii) Echo
6. Other investigation :-
i) Myocardial perfusion imaging
ii) Cardiac MRI with Gadolinium enhancement
iii) Gallium 67 scans
iv) FDG PET
PANDA SIGN & LAMBDA SIGN IN GALLIUM 67 SCAN IN SARCOIDOSIS
 Non caseating granuloma- Lung
Courtesy : Dr. Ajit Nambiar, HOD, Pathology, ASM
 Non caseating granuloma- spleen (low power)
Courtesy : Dr. Ajit Nambiar, HOD, Pathology, ASM
 Nervous System Manifestations

1. Unilateral VII nerve palsy (most common

manifestation)
2. Leptomeningeal involvement (25%)
3. Other cranial neuropathies
4. Hypothalamic and pituitary lesion
5. Psychiatric and cognitive dysfunction
6. Mononeuritis multiplex
7. Seizure
 Other Manifestations

1. Bone marrow involvement  15 to 40 %

2. Splenomegaly  5 to 10 %
3. Asymptomatic liver involvement – 60 to 90 %
4. Cholestatic liver involvement – 20 to 30%
5. Kidney – rarely affected
6. Lofgren’s syndrome :- i) Erythema Nodosum
ii) Arthritis
iii) Hilar Adenopathy
7. Heerfordt-Waldenström syndrome which constitutes of symptoms :-
i) Fever
ii) Parotid enlargement
iii) Anterior Uveitis
iv) Facial Palsy
Musculoskeletal Involvement
1. Bony involvement seen in 3-13% cases  involves hands and
feet
2. X-Rays – Radiographic Osseous disease
3. Osteopenia and Osteoporosis can occur as a result of steroid use
4. Acute Sarcoid Arthritis
 Symmetric polyarthritis
 First feet  ankles  later knees  PIP  MCP  Wrist
Elbows
 Symmetric ankle arthritis ( characteristically seen )
Sensitivity 95 %
Specificity 92 %
 Dactylitis can occur
 Remember MCQ – Dactylitis seen in i) Psoriatic Arthritis
ii) Reactive Arthritis
iii) Sarcoidosis
5. Myopathy in 5 % of patients
Childhood Sarcoidosis
1. Rare in children
2. Older Children have disease like adults –
lymphadenopathy and pulmonary involvement
3. <5 years – younger children have triad i) Rash 80% ii)
Uveitis iii) Arthritis
In children, slit lamp examination of eye is mandatory as
choroidal and conjunctival granulomas can occur

Differential Diagnosis :-
1) Juvenile idiopathic arthritis
2) Lymphomas
3) Infectious disease like TB
Treatment
1) Steroids
2)Methotrexate
3)HCQS more useful for skin
4)NSAIDS- joints

* Prognosis is better than adults

Assessment of Sarcoidosis
1. History and physical examination ( Environmental and occupational exposure,
family history )
2. Biopsy of affected organ, lymph nodes
3. X- Ray chest PA and lateral
4. PFT – TLC (Total lung capacity), DLCO
5. ECG
6. Complete eye check up
7. CBC, Platelet counts, serum calcium, Creatinine, S. Alkaline Phosphatase,
AST/ALT.
8. Serum ACE levels
9. 24 hours urine calcium

Other tests
10. Heart – Holter, Echo, PET MRI, EPS
11. Lung – Right heart catheterization for pulmonary hypertension.
12. CNS – MRI, CSF analysis
Investigation :-
1) Bronchoscopy and PFT’s
2) Transbronchial biopsy (EBUS)
3) Kveim-siltzbach skin test (rarely done)
4) HRCT – very useful in ILD

• Remember – Non caseating granulomas, anergy ( Negative

Mantoux’s test), Bilateral hilar lymphadenopathy, increased ACE
levels and increased calcium favours sarcoidosis

• In unclear cases, rule out

1) Infections – TB, Cryptococcosis, Aspergillosis, Histoplasmosis,
Coccidiodomycosis, Pneumocystis jiroveci pneumonia, Mycoplasmosis,
Pneumoconiosis and Wegeners granulomatosis
2) Lymph node – TB, Brucellosis, Toxoplasmosis, Kikuchis disease, Cat
Scratch disease, Hodgkins lymphoma, Non Hodgkins lymphoma
3) Skin- TB, Fungal infection, Atypical mycobacteria
4) Liver – rule out i) TB ii)Brucellosis iii) Schistosomiasis iv) Primary
biliary cirrhosis v) Crohn’s disease vi) Hodgkins disease vii) NHL
Treatment of Sarcoidosis

1) Steroids – oral and inhaled

2) DMARDS like methotrexate and HCQS
3) Complete heart block  Pacemakers and AICD’s
4) Joints  NSAIDS
5) Eye  Local steroid
Adverse Prognostic Indicators
a)Age of onset
b)Black race
c) Cardiac involvement
d) Neurological involvement
e) Lupus Pernio
f) Splenomegaly

Good Prognostic Indicators

a)Erythema Nodosum
b) Lofgren’s Syndrome
THANK YOU
Dr. Col. Vishal Marwaha