Internal Diseases and Therapy · MU Sofia

50. ANCA associated vasculitis

Covers the three ANCA-associated small vessel vasculitides: granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. For granulomatosis with polyangiitis it sets out the three pathological hallmarks, nasal, ocular and renal features, and the usual renal presentation. It then describes microscopic polyangiitis, its tropism for kidneys and lungs, and the phases of eosinophilic granulomatosis, ending with treatment.

Inside: 1 section · 1 labelled picture · 16 flashcards · 2 min

Topic 50 of 139 in the MU Sofia syllabus for Internal Diseases and Therapy (Medicine, Years 4 and 5). The free AceMed note covers it in 1 section, about 366 words (2 min of reading), with 16 flashcards.

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  1. ANCA associated vasculitis

ANCA associated vasculitis

These are the Antineutrophil cytoplasmic antibody (ANCA) associated vasculitides. They are a group of small vessel vasculitis. Within this group there are three main diseases:

  • Microscopic polyangiitis
  • Granulomatosis with polyangiitis (formerly Wegener granulomatosis)
  • Eosinophilic granulomatosis with polyangiitis (aka Churg-Strauss syndrome)

ANCAs are antibodies that are directed against the granules within neutrophils and monocytes. They attack these cells and amplify the inflammatory process that is already occurring. Lets take a look at these diseases.

Image: “C ANCA” by Simon Caulton, CC BY-SA 3.0, via Wikimedia Commons.

Granulomatosis with polyangiitis

Known as GPA for short, this involves the upper respiratory tract, lungs and kidneys. There are three pathological hallmarks of GPA, these are:

  1. 1. Granulomatous inflammation in the upper or lower respiratory tract
  2. 2. Necrotising vasculitis affecting arteries or veins
  3. 3. Segmental glomerulonephritis associated with necrosis and thrombosis of capillary loops, with or without granulomatous lesions

90% of patients have nasal involvement with crusting or bleeding from the nose, often with nasal septal perforation and collapse of the nasal bridge leading to the saddle nose deformity. Conductive and sensorineural hearing loss can also occur due to lesions of the middle ear. Orbital masses, scleritis, and peripheral ulcerative keratitis are the most dangerous ocular lesions. However, the most common clinical presentation is a rapidly progressive glomerulonephritis with hematuria, red blood cell casts and proteinuria. Many patients also present with arthralgia.

Microscopic polyangiitis

Characterised by:

  1. 1. Non Granulomatous necrotising vasculitis with few or no immune deposits
  2. 2. Involvement of small blood vessel in the arterial or venous circulation
  3. 3. Tropism for the kidneys and lungs

Both of these diseases can be treated with a combination of rituximab and glucocorticoids.

Eosinophilic granulomatosis with polyangiitis

This is an eosinophil rich form of granulomatous inflammation that involves the respiratory tract and other organs. The two hallmarks are asthma and eosinophilia. There are various phases of the disease described:

  1. 1. Prodromal phase with asthma or allergic rhinitis that may last months or years
  2. 2. Eosinophilia - tissue infiltration phase where eosinophils are observed in lungs, GI tract and other tissue
  3. 3. A vasculitic phase where systemic necrotising vasculitis afflicts many organs from the heart and lungs to peripheral nerves and skin

Treatment is prednisolone.

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