CHURG- STRAUSS SYNDROME (Eosinophilic Granulomatosis with Polyangiitis (EGPA)
Churg & Strauss is characterized by asthma, eosinophilla, extravascular granuloma formation, vasculitis.
Histophysiological features–
A characteristic feature is eosinophilic granulomatosis with polyangitis.
p- ANCA is an antibody associated.
Clinical Features-
Fever, malaise, anorexia, weight loss.
Mononeuritis multiplex is the second most common features.
Allergic rhinitis and sinusitis.
Asthma
Peripheral and tissue eosinophillia, extravascular necrotizing granuloma.
Striking eosinophilla- >1000/ cells
Diagnosis-
ESR elevated
Α2– globulins found
Treatment-
Glucocorticoids
In case of fail glucocorticoids treatmet, the treatment of choice is a combined of daily cyclophosphamide and prednisone.
Exam Question
Asthma ,Granuloma, Perpheral eosinophilia, Glomerulonephritis, Multisystem involvement of vessels are seen in churg- strauss syndrome
Mononeuritis multiplex ,regular attacks of bronchial asthma preceded by allergic rhinitis, cutaneous purpura with eosinophilia is suggesitve of churg- strauss syndrome
Lung is characteristically involved in churg- strauss syndrome
Churg Strauss Syndrome is associated with pulmonary eosinophillic pneumonia
Churg Strauss Syndrome is small vessels vasculitis
Mononeuritis multiplex is common in Churg Strauss Syndrome
Churg Strauss Syndrome is Often associated with Allergic Rhinitis
Lower Respiratory Tract involvement is more frequently seen in Churg Strauss Syndrome in comparison to Wegener’s Granulomatosis
Don’t Forget to Solve all the previous Year Question asked on Churg-Strauss Syndrome
Intravascular granulomas REF: Harrison 17th ed chapter 319
Churg-Strauss syndrome, also referred to as allergic angiitis and granulomatosis, was described in 1951 by Churg and Strauss and is characterized by asthma, peripheral and tissue eosinophilia, extravascular granuloma formation, and vasculitis of multiple organ systems.
Q. 2
Glomerulonephritis is NOT a feature of:
A
Granulomatosis with polyangiitis (Wegener’s)
B
Churg-Strauss syndrome
C
Microscopic polyangiitis
D
Polyarteritis nodosa
Q. 2
Glomerulonephritis is NOT a feature of:
A
Granulomatosis with polyangiitis (Wegener’s)
B
Churg-Strauss syndrome
C
Microscopic polyangiitis
D
Polyarteritis nodosa
Ans.
D
Explanation:
In Polyarteritis nodosa the pathology in the kidney is that of arteritis without glomerulonephritis.
In patients with significant hypertension, typical pathologic features of glomerulosclerosis may be seen.
Ref: Harrisons principles of internal medicine, 18th edition, Page: 2794.
Q. 3
A 45 year old lady presented with features suggestive of mononeuritis multiplex. She gives a h/o regular attacks of bronchial asthma preceded by allergic rhinitis. She also has cutaneous purpura. Blood routine examination revealed eosinophilia. The diagnosis to be considered in this clinical scenario is?
A
Microscopic polyangiitis
B
Endogenous bronchial asthma
C
Churg strauss syndrome
D
Wegeners vasculitis
Q. 3
A 45 year old lady presented with features suggestive of mononeuritis multiplex. She gives a h/o regular attacks of bronchial asthma preceded by allergic rhinitis. She also has cutaneous purpura. Blood routine examination revealed eosinophilia. The diagnosis to be considered in this clinical scenario is?
A
Microscopic polyangiitis
B
Endogenous bronchial asthma
C
Churg strauss syndrome
D
Wegeners vasculitis
Ans.
C
Explanation:
When small-vessel vasculitis is associated with peripheral eosinophilia, cutaneous purpura, mononeuritis, asthma, and allergic rhinitis, a diagnosis of Churg-Strauss syndrome is considered.
Ref: Harrisons Principles of Internal Medicine, 18th Edition, Page 2344
Q. 4
Granuloma is seen in all except –
A
Giant cell arteritis
B
Microscopic polyangitis
C
Wegner’s granulomatosis
D
Churg-strauss disease
Q. 4
Granuloma is seen in all except –
A
Giant cell arteritis
B
Microscopic polyangitis
C
Wegner’s granulomatosis
D
Churg-strauss disease
Ans.
B
Explanation:
Ans. is ‘b’ i.e., Microscopic polyangitis
In microscopic polyangitis, granuloma are absent.
o There are two types of inflammation in the wall of vessels in systemic vasculitis ‑
I. Granulomatous inflammation —> characterized by formation of granuloma in vessel wall.
2. Necrotizing inflammation characterized by fibrinoid necrosis in the vessel wall.
Vasculitis with granulomatous inflammation o Giant cell arteritis
o Takayasu’s disease
o Wegner’s granulomatosis
o Churg strauss syndrome
Vasculitis with necrotizing inflammation
Polyarteritis nodosa
Churg strauss syndrome
Wegner’s granulomatosis
Microscopic polyangitis
Note – Wegner’s granulomatosis and churg strauss syndrome have feature of both granulomatous inflammation (predominant) and necrotizing inflammation.
Q. 5
Lung is characteristically involved in –
A
Churg-Strauss syndrome
B
HSP
C
PAN
D
ITP
Q. 5
Lung is characteristically involved in –
A
Churg-Strauss syndrome
B
HSP
C
PAN
D
ITP
Ans.
A
Explanation:
Ans. is ‘a’ i.e., Churg-Strauss syndrome
Churg- strauss syndrome
o Also known as allergic granulomatosis and angitis.
o It is a medium and small size vasculitis, invoves mainly the blood vessels of lungs, GIT, peripheral nerves, heart, skin, kidney.
o As it is allergic, vasculitis it is associated with eosinophilia and asthma.
o It is granulomatous necrotizing vasculitis.
o It is associated with p-ANCA.
Stages
This disease has three distinct stages ‑
o Stage l —> Involves the sinuses and the onset of allergies —> nasal polyp, allergic rhinitis.
o Stage 2 —> Acute asthma occurs.
o Stage 3 —> The third and final stage involves the various organ systems –
1. Peripheral nervous system —> Mononeuritis multiplex with severe neural pain.
Eosinophilic granuloma (pulmonary histiocytosis X or Langerhans cell granulomatosis).
Q. 7
All of the following are small vessels vasculitis, Except:
A
Classical PAN
B
Wegner’s granulomatosis
C
HSP
D
Churg Strauss Syndrome
Q. 7
All of the following are small vessels vasculitis, Except:
A
Classical PAN
B
Wegner’s granulomatosis
C
HSP
D
Churg Strauss Syndrome
Ans.
A
Explanation:
Answer is A (Classical PAN):
Classical PAN is a predominantly medium vessel vasculitis and not a small vessel vasculitis.
Q. 8
ANCA positive vasculitis include all of the following Except:
A
Wegner’s granulomatosis
B
Churg strauss syndrome
C
Microscopic PAN
D
Good pasture’s syndrome
Q. 8
ANCA positive vasculitis include all of the following Except:
A
Wegner’s granulomatosis
B
Churg strauss syndrome
C
Microscopic PAN
D
Good pasture’s syndrome
Ans.
D
Explanation:
Answer is D (Good pasture’s syndrome):
Goodpasture’s syndrome is not classified as an ANCA positive vasculitis.
Q. 9
Which of the following is not true about Churg Strauss Syndrome
A
Asthma
B
Peripheral eosinophilia
C
Vasculitis of multiple organ systems
D
Intravascular Granulomas
Q. 9
Which of the following is not true about Churg Strauss Syndrome
A
Asthma
B
Peripheral eosinophilia
C
Vasculitis of multiple organ systems
D
Intravascular Granulomas
Ans.
D
Explanation:
Answer is D (Intravascular Granulomas):
Churg Strauss Syndrome is typically charachterized by extravascular granulomas.
`Churg Strauss Syndrome also referred to allergic angitis and granulomatosis is charachterized by asthma, peripheral and tissue eosinophilia, extravascular granuloma formation and vasculitis of multiple organ systems ‘- Harrison’s 18th
`Churg Strauss Syndrome is typically associated with Cutaneous Extravascular Necrotizing Granulomas (CENG)’. – Practical Immunodermatology
Note that intravascular granulomas present within the walls of the vessels themselves may also be seen in Churg Strauss Syndrome. This is however not a charachteristic feature.
Q. 10
All of the following statements about Churg Strauss Syndrome are true, except:
A
Marked Eosinophilia
B
Mononeuritis multiplex is common
C
Renal failure is common
D
Often associated with Allergic Rhinitis
Q. 10
All of the following statements about Churg Strauss Syndrome are true, except:
A
Marked Eosinophilia
B
Mononeuritis multiplex is common
C
Renal failure is common
D
Often associated with Allergic Rhinitis
Ans.
C
Explanation:
Answer is C (Renal failure is common):
Churg Strauss Syndrome is not prominent and renal failure is rare.
The Renal disease in Churg Strauss Syndrome is less common and generally less severe than with granulomatosis with polyangitis (Wegener’s) and microscopic polyangitis ‘- Harrison’s 18th/2793
‘Renal failure is rare in Churg Strauss syndrome – Pulmonary Involvement in Systemic Autoimmune Disease
Q. 11
Which of the following is more frequently seen in Churg Strauss Syndrome in comparison to Wegener’s Granulomatosis
A
Renal involvement
B
Lower Respiratory Tract involvement
C
Eye involvement
D
Upper Respiratory Tract involvement
Q. 11
Which of the following is more frequently seen in Churg Strauss Syndrome in comparison to Wegener’s Granulomatosis
A
Renal involvement
B
Lower Respiratory Tract involvement
C
Eye involvement
D
Upper Respiratory Tract involvement
Ans.
B
Explanation:
Answer is B (Lower Respiratory Tract involvement):
Lower Respiratory Tract involvement is typically more common in Churg Strauss Syndrome in comparison to Wegener’s Granulomatosis.
Organs involved in small vessels vasculitis:
Organ involvement throughout disease course
Wegner’s granulomatosis
Microscopic polyangitis
Churg-Strauss syndrome
Upper respiratory tract
80%
Uncommon
55%
Lower respiratory tract
70%
30%
100%
Eye
46%
10%
6%
Kidney
80%
90%
33%
Peripheral nervous system
35%
20%
67%
Gastrointestinal system
5%
5%
17%
Heart
Uncommon
Uncommon
28%
Skin
50%
25%
44%
Constitutional symptoms
95%
90%
94%
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