Tag: Churg-Strauss Syndrome

Intravascular granulomas REF: Harrison 17^th 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.

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.

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

Ans. is ‘b’ i.e., Microscopic polyangitis

Note – Wegner’s granulomatosis and churg strauss syndrome have feature of both granulomatous inflammation (predominant) and necrotizing inflammation.

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.

2.       Skin         -3 Palpable purpura, papules, ulcer, vesiculobillous lesion.

3.       Heart –> CHF

4.       Kidney         Pauciimmune glomerulonephritis.

5.       Joints —> Migratory arthritis.

6.       Ear               Conductive deafness.

7.      There is eosinophilia and raised IgE as occur with allergic conditions.

8.       Lung —> Nonfixed migratory pulmonary infiltrates.

Answer is D (Wegener’s granulomatosis)

Pulmonary Eosinophilia

Extrinsic eosinophilic syndromes:

• Loeffler syndrome
• DRESS syndrome: The Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome Parasitic infections
• Schistosomiasis
• TPE: Tropical Pulmonary Eosinophilia.
• Bronchocentric granulomatosis
• Fungal causes: Allergic bronchopulmonary aspergillosis (ABPA)
• Strongyloidiasis

Intrinsic pulmonary eosinophilic syndromes:

• Chronic eosinophilic pneumonia
• Idiopathic hypereosinophilic syndrome
• Churg-Strauss syndrome
• Eosinophilic granuloma (pulmonary histiocytosis X or Langerhans cell granulomatosis).

Answer is A (Classical PAN):

Classical PAN is a predominantly medium vessel vasculitis and not a small vessel vasculitis.

Answer is D (Good pasture’s syndrome):

Goodpasture’s syndrome is not classified as an ANCA positive vasculitis.

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.

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

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: