WHEATON • ROCKVILLE • CHEVY CHASE • WASHINGTON, DC in a Nutshell

By Justin Peng MD, FACR Arthritis and Rheumatism Associates, P.C.

ARTHRITIS Vasculitis literally means inflammation • Medium sized vessel vasculitidies of the blood vessels. It is defined by include AND the presence of inflammatory (PAN), , and RHEUMATISM leukocytes in the vessel walls resulting primary CNS Vasculitis. ASSOCIATES, P.C. in reactive damage and loss of vessel • Small vessel vasculitidies include integrity which can lead to rashes, Wegener’s (now named bleeding, ischemia or necrosis. Granulomatosis with Polyangiitis Board Certified Rheumatologists Vasculitidies are rare and can be a or GPA), , Herbert S.B. Baraf primary process, or secondary to Churg-Strauss, which are ANCA MD FACP MACR another underlying disease or associated. Henoch-Schönlein Robert L. Rosenberg autoimmune condition. They can , , MD FACR CCD affect people of any age, but some and Evan L. Siegel types occur more in certain age Leukocytoclastic Vasculitis, and MD FACR groups and ethnicities. Classically, they secondary to conditions like Emma DiIorio are categorized by the predominant and . MD FACR sizes of the blood vessels that are Vasculitis can also be secondary to David G. Borenstein affected, and also by the presence or medications, or infections like MD MACP MACR absence of antineutrophil cytoplasmic Hepatitis B and C, HIV, CMV, Alan K. Matsumoto antibodies (ANCA). EBV, Parvovirus. MD FACP FACR • Large vessel vasculitidies include David P. Wolfe MD FACR Takayasu Arteritis and Giant Cell Symptoms are extremely varied, Arteritis (GCA), or Temporal depending on the cause and which Paul J. DeMarco MD FACP FACR Arteritis (which occurs in about organ is involved; they can range 15% of those with Polymyalgia from very mild to quite severe. Shari B. Diamond MD FACP FACR Rheumatica). (over) Ashley D. Beall MD FACR Takayasu’s arteritis Angus B. Worthing MD FACR Temporal artery Guada Respicio MD MS FACP FACR Ophthalmic artery Justin Peng Occipital artery MD FACR Facial artery

Rachel Kaiser Lingual artery MD MPH FACP FACR Nicole Saddic Thomas MD FACR CENTRAL CALL CENTER 301.942.7600 www.washingtonarthritis.com ARTHRITIS AND RHEUMATISM ASSOCIATES, P.C. Board Certified Rheumatologists

For example, Cutaneous useful tests sometimes include EMG/ Leukoytoclastic Vasculitis affects the NCS (electromyography/nerve smallest blood vessels and can cause conduction studies), imaging including rashes including palpable purpura (see MRI/MRA or ultrasound, below), nodules or ulcers. On the arteriography, and most definitively: other hand, and Tissue Biopsy. Takayasu’s arteritis affects the larger arteries and can cause more serious Treatment depends on the nature, neurological and cardiovascular severity and type of vasculitis, as well symptoms. The presence of vasculitis as what organs are affected and to should be considered with systemic what degree they are affected. For symptoms and evidence of single and/ example, in mild Hypersensitivity or multiorgan dysfunction. vasculitis due to a drug, discontinuing Disease outcomes vary depending on the offending drug may be adequate, the type of vasculitis. It is usually good Diagnostic evaluation should also along with antihistamines and for patients with Hypersensitivity consider age and gender, a detailed observation. However, for other vasculitis, Giant Cell Arteritis, or history, including drug use, and vasculitidies that cause pulmonary, Henoch-Schönlein purpura. On infectious disease exposures. General neurologic, or renal manifestations, the other hand, some forms of labs should be obtained to identify aggressive treatment may be required, Polyarteritis Nodosa and Wegeners which type of disorder, and the degree including corticosteroids (Prednisone), Granulomatosis have five-year survival of organ involvement if any. and immunosuppressive therapies rates of 60-70 percent respectively. Additional testing can be directed by a including Methotrexate, Azathioprine, Fortunately, there are effective rheumatologist, and depending on Cyclophosphamide and Rituximab. therapies currently and ongoing pretest probability can include ANA The goal is to induce remission, to research has provided promising results (Anti-nuclear antibodies), complement minimize drug toxicity and to in sustaining remission even among levels, ESR, CRP, and ANCA. Other monitor for signs of recurrence. those with serious disease. n

PHOTO SOURCE: AMERICAN COLLEGE OF RHEUMATOLOGY

CENTRAL CALL CENTER 301.942.7600 www.washingtonarthritis.com