**2. Classification**

American College of Rheumatology (ACR) presented the classification criteria for vasculitis in 1990 (**Table 1**). According to this criteria vasculitis was classified into primary and secondary types. Both these types were dependent on the size of vessel involved. Vasculitis affecting the large arteries include giant cell arteritis (GCA) and Takayasu arteritis. Medium vessel vasculitis includes polyarteritis nodosa (PAN) and Kawasaki disease, while small vessel vasculitis contains granulomatosis with polyangiitis (GPA) formerly known as Wegener's granulomatosis (WG), Churg-Strauss syndrome now known as eosinophilic granulomatosis with polyangiitis (EGPS), microscopic polyangiitis (MPA), Henoch Schonlein purpura and cryoglobulinemia. Secondary vasculitides encompass vasculitis secondary to rheumatoid arthritis and various infections (bacterial, viral and fungal) [3].

Infections affecting large arteries commonly include *Staphylococcus*, *Salmonella*, *Streptococcus*, coccidioidomycosis and treponema pallidum. Hepatitis B and C virus along with human immunodeficiency virus and Parvovirus B19 commonly affects medium sized vessels. Possible mechanism for infection related vasculitis includes (a) direct microbial invasion and (b) immune-mediated process either by humoral or cellular responses. Hepatitis B virus has firmly been seen with polyarteritis nodosa for more than 30 years. In France, the declining rate of hepatitis B infection has correlated with falling levels of hepatitis B-associated polyarteritis nodosa. There is a strong association between hepatitis C virus and mixed essential cryoglobulinemia. Vasculitis has been identified as a rare manifestation of human immunodeficiency virus. Multiple patterns have been described including polyarteritis nodosa, hypersensitivity vasculitis and large vessel disease [3].

Course of rheumatoid arthritis can be complicated by medium to small vessel vasculitis. This is common in men and linked with positive RA factor. It may present in a variety of ways including distal arteritis, splinter hemorrhages, peripheral neuropathy with mononeuritis multiplex and aortitis [4].

In year 2012 the International Chapel Hill Consensus conference adopted names for vasculitis on nomenclature of vasculitides as shown in **Table 2**. This classified vasculitis not only according to the size of vessel involved (as in ACR criteria) but also included a few other subtypes mainly, variable vessel vasculitis, single organ vasculitis, vasculitis associated with systemic diseases and vasculitis associated with probable etiology [5].


**159**

**3.1 Rituximab**

**Table 2.**

*Treatment of Vasculitis: Beyond the Basics DOI: http://dx.doi.org/10.5772/intechopen.92729*

Anti-neutrophil cytoplasmic antibody-associated vasculitis

**Large vessel vasculitis** Takayasu's arteritis Giant cell arteritis

**Medium vessel vasculitis** Polyarteritis nodosa Kawasaki disease **Small vessel vasculitis**

Microscopic polyangiitis

Granulomatosis with polyangiitis

Cryoglobulinemic vasculitis

Variable vessel vasculitis

Behcet's disease

Lupus vasculitis Rheumatoid vasculitis Sarcoid vasculitis

Eosinophilic granulomatosis with polyangiitis Immune complex small vessel vasculitis

IgA vasculitis (Henoch Schonlein purpura)

Vasculitis associated with systemic disease

Vasculitis associated with probable etiology Hepatitis C-associated cryoglobulinemic vasculitis

Drug-associated immune complex vasculitis Drug-associated ANCA-associated vasculitis

Hepatitis B-associated vasculitis

*Modern Chapel Hill classification [5].*

A wide range of drugs have been reported to cause a vasculitic reaction. ANCA-associated vasculitis has been attributed to use of various drugs including Hydralazine, Propylthiouracil, Allopurinol and Sulfasalazine. Leukotriene receptor antagonist, Montelukast and Zafirlukast, have been linked to Churg-Strauss syndrome. Acute vasculitis may be the presenting feature of an undiagnosed malignancy. Common malignancies associated with vasculitis are myelodysplasia,

It is a monoclonal antibody, which is directed against CD20 that is expressed on developing B cell. Although not clearly understood, this antibody can induce apoptosis of these developing B cells. In April 2011, Rituximab was the first agent to

lymphoma and multiple myeloma [3].

**3. Treatment with biological agents**

be approved by FDA for the treatment of vasculitis [6].

#### **Table 1.**

*ACR classification of vasculitis [3].*

*Vascular Biology - Selection of Mechanisms and Clinical Applications*

**2. Classification**

we discuss the treatment of vasculitis beyond the prototype drugs (cyclophospha-

American College of Rheumatology (ACR) presented the classification criteria for vasculitis in 1990 (**Table 1**). According to this criteria vasculitis was classified into primary and secondary types. Both these types were dependent on the size of vessel involved. Vasculitis affecting the large arteries include giant cell arteritis (GCA) and Takayasu arteritis. Medium vessel vasculitis includes polyarteritis nodosa (PAN) and Kawasaki disease, while small vessel vasculitis contains granulomatosis with polyangiitis (GPA) formerly known as Wegener's granulomatosis (WG), Churg-Strauss syndrome now known as eosinophilic granulomatosis with polyangiitis (EGPS), microscopic polyangiitis (MPA), Henoch Schonlein purpura and cryoglobulinemia. Secondary vasculitides encompass vasculitis secondary to rheumatoid arthritis and various infections (bacterial, viral and fungal) [3].

Infections affecting large arteries commonly include *Staphylococcus*, *Salmonella*,

Course of rheumatoid arthritis can be complicated by medium to small vessel vasculitis. This is common in men and linked with positive RA factor. It may present in a variety of ways including distal arteritis, splinter hemorrhages, peripheral

In year 2012 the International Chapel Hill Consensus conference adopted names for vasculitis on nomenclature of vasculitides as shown in **Table 2**. This classified vasculitis not only according to the size of vessel involved (as in ACR criteria) but also included a few other subtypes mainly, variable vessel vasculitis, single organ vasculitis, vasculitis associated with systemic diseases and vasculitis associated with

> Aortitis associated with rheumatoid arthritis, infections

Hepatitis B-associated, polyarteritis nodosa

Vasculitis secondary to rheumatoid arthritis, drugs

infections

Drugs, hepatitis C associated,

*Streptococcus*, coccidioidomycosis and treponema pallidum. Hepatitis B and C virus along with human immunodeficiency virus and Parvovirus B19 commonly affects medium sized vessels. Possible mechanism for infection related vasculitis includes (a) direct microbial invasion and (b) immune-mediated process either by humoral or cellular responses. Hepatitis B virus has firmly been seen with polyarteritis nodosa for more than 30 years. In France, the declining rate of hepatitis B infection has correlated with falling levels of hepatitis B-associated polyarteritis nodosa. There is a strong association between hepatitis C virus and mixed essential cryoglobulinemia. Vasculitis has been identified as a rare manifestation of human immunodeficiency virus. Multiple patterns have been described including polyar-

teritis nodosa, hypersensitivity vasculitis and large vessel disease [3].

**Dominant vessel Primary Secondary**

Granulomatosis with polyangiitis, Churg-Strauss syndrome, microscopic polyangiitis

neuropathy with mononeuritis multiplex and aortitis [4].

Takayasu arteritis

Kawasaki disease

Cryoglobulinemia

mide, azathioprine, mycophenolate mofetil, etc.), with biological agents.

**158**

**Table 1.**

probable etiology [5].

Small vessels and medium arteries

*ACR classification of vasculitis [3].*

Large arteries Giant cell arteritis

Medium arteries Classic polyarteritis nodosa

Small vessels Henoch-Schonlein purpura


#### **Table 2.**

*Modern Chapel Hill classification [5].*

A wide range of drugs have been reported to cause a vasculitic reaction. ANCA-associated vasculitis has been attributed to use of various drugs including Hydralazine, Propylthiouracil, Allopurinol and Sulfasalazine. Leukotriene receptor antagonist, Montelukast and Zafirlukast, have been linked to Churg-Strauss syndrome. Acute vasculitis may be the presenting feature of an undiagnosed malignancy. Common malignancies associated with vasculitis are myelodysplasia, lymphoma and multiple myeloma [3].
