Dr.

Vaishnavi
 Sarcoidosis is an inflammatory disease
characterized by the presence of non
caseating granulomas
 Multisystem involvement and diagnosis
requires involvement of two or more organs
 Most commonly involved organs are
 Lungs / Liver / Skin / Eye
 Most likely – An infectious or non infectious
environmental agent that triggers an
inflammatory response in a genetically
susceptible host
 Possible infectious agents - Propionibacter
acnes and Mycobacterium tuberculosis or
similar organism
 Environmental exposure to insecticides and
moulds
 Health care workers
 Represents particular host response to
multiple agents
 Highest in Nordic population
 More in African Americans than whites
 Women more than men
 Occurs in young and otherwise healthy adults
 5 percent cases have family history of
Sarcoidosis
 Granuloma is the pathologic hallmark of
Sarcoidosis
 There is a local accumulation of inflammatory
cells – influx of T- helper cells – activated
monocytes
 The HLA-CD4 complex – APC’s present an
unknown antigen to T helper cell –increased
release of several cytokines including IL-2 ( T
cell),IFN Gamma and TNF ( macrophage)
 Persistent form of disease is associated with
high levels of IL 8 and TNF secretion .
 Asymptomatic to Organ failure
 Pt presents to OPD with complaints of cough
and dyspnoea for 2 to 4 weeks – CXR aids in
diagnosis
 Fatigue,fever,night sweats ,weight loss
 Patients with cutaneous manifestations are
more likely to be diagnosed ( erythema
nodosum,lupus pernio etc)
 Occurs in > 90% of cases ( detected by CXR-
BILATERAL HILAR ADENOPATHY)
 Stage 1 : Hilar adenopathy alone
 Stage 2 : Combination of adenopathy and
infiltrates
 Stage 3 : Infiltrates alone (the upper lobe )
predominanty
 NON INFECTIOUS
 Hypersensitivity pneumonitis
 Silicosis
 Langerhans cell histiocytosis
 INFECTIOUS
 Tuberculosis
 Pneumocystis pneumonia
 Present in 1/3 rd of sarcoidosis patients
 Erythema nodosum
 Maculopapular lesions
 Hypo and hyeper pigmentation
 Keloid formation
 Subcutaneous nodules
 Lupus pernio - Bridge of nose,area between
the eyes and cheeks – purplish papules –
often indurated – skin biopsy is diagnostic
 Hilar adenopathy
 Uveitis
 Erythema nodosum
 More common in Japanese
 Symptoms : Photophobia , increased
tearing/dry eyes and blurred vision
sometimes blindness
 Most common manifestation is anterior
uveitis
 Posterior – retinitis and pars planitis
 Regular ophthalmic examination is a must
 Liver involvement is seen in ½ of the sarcoidosis
cases
 LFT – shows elevation of Alkaline phosphatase
consistent with obstructive pattern
 Elevated transaminases can also occur
 Can present as
 Hepatomegaly
 PHTN
 Ascites
 Oesophageal varices
 Avoid interferon therapy as it worsens disease
 Lymphopenia – due to sequestration of
lymphocytes into areas of inflammation
 Anemia
 Bone marrow examination shows granulomas
 Splenomegaly in 5 to 10 %
 Splenectomy indicated in massive
symptomatic splenomegaly and severe
pancytopenia
 Direct kidney involvement in <5% of
sarcoidosis patients – granuloma in kidney –
nephritis
 Hypercalcemia is more likely cause of renal
disease leading to renal failure
 Both hypercalcemia and hypercalciuria can
occur in 10 % patients.
 More common in white men
 Increased 1,25 dihydroxyvitamin D by the
granuloma itself causing increased intestinal
absorption of calcium – suppressed PTH
 Serum calcium levels should be looked for
always and repeated after sun exposure and
in the summer months.
 24 hr urine calcium measurement is required
 Any part of the central and peripheral
nervous system can be affected
 Most commonly
 cranial nerve involvement
 Basilar meningitis
 Myelopathy
 Anterior hypothalamic disease with diabetes
insipidus
 Sometimes MRI contrast may show SOL
 Seizures
 Cognitive disorders
 Seventh nerve palsy
 Optic neuritis
 It is more common in Japanese than other
races
 Presents as the following due to diffuse
infiltration by the granulomas and pressure
effects
 Congestive cardiac failure
 Cardiac arrhythmias
 Reduced ejection fraction
 AV node infiltration leading to heart block
 Ventricular arrhythmias and VT are reasons
for sudden death
 Implanted defibrillator
 Systemic therapy – takes 6 months or more to
act…occurs whenever medications are
stopped
 Granulomatous involvement of bone and
muscle can be documented bu xray,MRI and
CT.
 Patient complaints of myalgia,arthralgia and
extreme fatigue
 Sarcoidosis is self limiting and non-life
threatening but organ threatening –
blindness, paraplegia,renal failure , lung
fibrosis etc are a few
 Death occurs mainly due to the organ
involvement and secondary infections eg:
mycetoma – leading to massive bleeding
 CXR – Assess lung involvement
 CT scan – Adenopathy >2 cm is calssic of
sarcoidosis.
 PET scan and CT – aid in selecting sites for
biopsies and extent of organ involved
 Cardiac PET scan in identifying extent of
cardiac sarcoidosis
 MRI with contrast in assessment of
extrapulmonary sarcoidosis
 Elevation of >50% of upper limit of ACE is
observed in sarcoidosis
 DD’s for increased ACE
 Leprosy
 Gaucher’s
 Hyperthyroidism
 Miliary tuberculosis
 Based on clinical features
 Uveitis,hypercalcemia,renal calculi,7th nerve
palsy,Erythema nodosum
 Kveim Siltzbach procedure : An intradermal
injection of specially prepared tissue from the
spleen of an known sarcoidosis patient - non
caseating granuloma develops at the site
 Tests : Skin biopsy
 Other organ biopsy
 BAL
 Elevated ACE Level
 Positive PET scan
 Glucocorticoids remain drug of choice
 Cutaneous sarcoidosis –
HCQ,Steroids,Minocycline
 Pulmonary and extra pulmonary –
Methotrxate,Azathioprine,Leflunomide,Mycophen
olate,Cyclophosphamide
 Biologic anti – TNF agents – Infliximab (MAB
against TNF )and Etanercept( TNF receptor
antagonist) and even Adalimumab to some
extent .Infliximab significantly improved lung
function ( contraindicated in persons with
tuberculosis)
THANK YOU