Sarcoidosi S: DR Avishek Naskar Asst Prof, Dept of Medicine, Esi Pgimsr, Joka
Sarcoidosi S: DR Avishek Naskar Asst Prof, Dept of Medicine, Esi Pgimsr, Joka
Sarcoidosi S: DR Avishek Naskar Asst Prof, Dept of Medicine, Esi Pgimsr, Joka
Dr Avishek Naskar
Asst prof, dept of Medicine,
ESI PGIMSR, Joka
WHAT IS IT
Sarcoidosis is a multisystem inflammatory disease of
unknown etiology that manifest as non-caseating
granulomas, predominantly in the lungs and intrathoracic
lymph nodes.
Incidence is 5 – 40 cases per 100,000 population, mostly
in white populations, also in people of African origin.
Sarcoidosis is misdiagnosed as TB in developing
countries like ours.
PATHOPHYSIOLOGY
There is no clear understanding of the whole
pathogenesis of sarcoidosis, but it is definitely known,
that CD4 T cells play a central role, and you will see that
they (CD4 T cells) are found abundantly, in tissues.
Pathogensis of Sarcoidosis. γ-IFN mainly stimulates granuloma formation. If
there is persistent action of the cytokine IL-8, then the disease progesses to
chronicity (fibrosis)
CLINICAL FEATURES
Asymptomatic - 5% cases.
Systemic complains (e.g. fever, weight loss, arthralgia,
night sweats) – 45%
Individual organs clinically…..
1)Lungs :
Cough & dyspnoea clinically
On investigations,
Hilar lymphadenopathy
Hepatomegaly
Anemia, lymphopenia
CLINICAL FEATURES CONTD…
6)Bones
Hypercalcemia – due to increased activity of 1α-
hydroxylase (Vit D activation increased)
8)Renal :
Granulomas in kidney and hypercalcemia, both may cause
renal impairment
9)Cardiac
Life threatening arrythmias – ventricular tachycardia, AV
nodal blocks
CHF (<10% cases)
CLINICALLY…
Lofgren’s syndrome : an acute form of Sarcoidosis with
erythema nodosum, hilar lymphadenopathy and uveitis.
Has good prognosis.
DIAGNOSIS
DIAGNOSIS CONTD…
The previous slide shows an algorithm for diagnosis of
Sarcoidosis
Classical syndrome means : An young or middle aged
lady with common clinical features , like erythema
nodosum, B/L hilar lymphadenopathy, uveitis,
hypercalcemia (on serum calcium level testing).
You should know the differences between TB and
sarcoidosis. TB is far common in India.
SARCOIDOSIS VS TB
TB can present in numerous forms in India, but subtle differences exist
with Sarcoidosis . Obviously, you should know TB better, but sarcoidosis
for theory too.
Any granuloma : Histiocytes fuse to form multinucleate giant cells,
epitheloid macrophages, lymphocytes, fibroblasts, with fibrosis. Difference
is the presence of caseation necrosis in centre of TB granuloma and its
absence in sarcoidosis.
TREATMENT
Treatment is mainly given for organ threatening disease
e.g. Uveitis/optic neuritis (may lead to blindness),
hypercalcemia, neurological manifestations.
But usually minor manifestations e.g. erythema nodosum
need no treatment or just symptomatic treatment.
For advanced lung fibrosis lung transplant
TREATMENT : ACUTE DISEASE
TREATMENT : CHRONIC SARCOIDOSIS
Thank you