Saturday, January 28, 2017

Churg Strauss Syndrome

Hello awesomites, I am kind of obsessed with fancy syndromes. So here is one of them.

Churg Strauss Syndrome (CSS) also known as Eosinophilic granulomatosis with polyangitis or allergic granulomatosis.
It is a rare  autoimmune condition, that causes inflammation of small and medium sized blood vessels.

Manifests in 3 stages-
Early stage (Prodromal stage) : Present as
Asthma or
Allergic Rhinitis
Sometimes with nasal polyps and sinusitis
(Remember 'A'  is the first letter, so it should always come first)

Second stage : Abnormally increased eosinophils= Hypereosinophilia
Which causes tissue damage mostly lung and digestive tract.
Manifestations are Night sweats, weight loss, cough, abdominal pain, GI bleeding, fever, purpura

Third stage: vasculitis- which leads to infarction which further leads to atrophy
Further progression leads to complications.
But not all patients develops all three stages, or progress in the same order.

Pathophysiology - Its a Autoimmune disorder where different cell types are responsible for immune response especially Eosinophils, T&B cells, endothelial and epithelial cells. Mainly it is Th2 mediated reaction.

Complications can be life threatening -
(Most Grievous)
M- Myocardial involvement is the most common complication and most common cause of death in CSS patients
G- GI bleeding, GI perforation, Glomerulonephritis, Glomerulosclerosis,
Granulomatous appendicitis

Treatment - Conventional treatment includes glucocorticoids like Prednisolone and immuno suppressive drugs like Azathioprine, cyclophosphamide.
Newer drugs direct against specific cytokines like mepolizumab have additional steroid sparing property angood tolerability. Use of  Rituximab is under investigation and limited to few cases.

That's all.  :)

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