Definition
A.Part of a group of disorders that result from inflammatory changes to walls of veins and arteries, causing damage to the mural structures, which ultimately may cause tissue ischemia and necrosis.
B.Nomenclature is changing for the specific forms of vasculitis based on the Chapel Hill Consensus Conference (CHCC).
C.Several different types.
1.Polyarteritis nodosa.
2.Kawasaki disease (more common in peds).
3.Buerger disease (thromboangiitis obliterans).
4.Primary angiitis of the central nervous system.
Incidence
A.Polyarteritis nodosa.
1.Not common: 30 cases per 1 million people.
2.Males affected more than females; common age presentation is in sixth decade.
B.Buerger disease (thromboangiitis obliterans).
1.Typical patient is a young, male smoker using raw tobacco.
C.Primary angiitis of the central nervous system.
1.Rare disorder with male predominance.
2.Can occur at any age, with median age being 50 years old.
Pathogenesis
A.Vasculitis can be a primary pathology or can be secondary to another underlying disease process.
B.Direct injury to the vessel, infectious agents, or immune processes are known to cause vasculitis.
C.Secondary vascular injury may occur from physical agents such as cold and irradiation, mechanical injuries, and toxins.
D.Polyarteritis nodosa.
1.First form of vasculitis reported.
2.Predilection for vessels of skin, peripheral nerves, mesenteric vessels, renal vessels, heart, and brain.
3.10% of cases caused by hepatitis B infection.
E.Buerger disease (thromboangiitis obliterans).
1.Highly cellular and inflammatory occlusive thrombus affecting the extremities.
F.Primary angiitis of the central nervous system.
1.Vasculitis affecting brain and spinal cord.
Predisposing Factors
A.Male gender.
B.Use of raw tobacco for Buerger disease.
Subjective Data
A.Common complaints/symptoms.
1.Polyarteritis nodosa.
a.Arthralgia, myalgia, and neuropathy.
b.Skin manifestations include lower extremity malleoli skin ulcerations (most common), digital gangrene, livedo reticularis, and subcutaneous nodules.
c.Acute abdomen presentation when abdominal vessels are affected including abdominal pain, nausea, and vomiting.
d.Hypertension when renal vessels are affected.
2.Buerger disease (thromboangiitis obliterans).
a.Distal extremity ischemia, ischemic digit ulcers, digit gangrene, migratory phlebitis.
3.Primary angiitis of the central nervous system.
a.Weeks to months of headaches, encephalopathy, and multifocal strokes.
B.Common/typical scenario.
1.Patients will complain of various symptoms upon presentation that may be mistaken for other diseases.
2.In polyarteritis nodosa, patients commonly present with acute abdominal pain.
3.In Buerger disease, patients present with pain in their extremities; in primary angiitis, patients may present with strokes.
C.Family and social history.
1.Buerger disease: Use of raw tobacco.
D.Review of systems.
1.Arthralgias.
2.Myalgias.
3.Paresthesias.
4.Skin changes.
5.Abdominal pain.
6.Nausea, vomiting.
7.Headaches.
8.Ataxia.
Physical Examination
A.Polyarteritis nodosa.
1.Ulcerations on malleoli.
2.Motor and sensory deficits.
3.Digital gangrene.
B.Kawasaki disease (more common in peds).
1.Bilateral conjunctivitis without exudate.
2.Lip and oral erythema.
3.Cervical lymphadenopathy.
4.Rash.
5.Edema of hands and feet.
C.Buerger disease (thromboangiitis obliterans).
1.Superficial phlebitis.
2.Digit ischemia.
D.Primary angiitis of the central nervous system.
1.Motor and sensory deficits.
Diagnostic Tests
A.Polyarteritis nodosa.
1.Anemia and leukocytosis.
2.Antineutrophil cytoplasmic antibodies (ANCA) negative.
3.Elevated C-reactive protein (CRP) and elevated erythrocyte sedimentation rate (ESR).
4.Hepatitis B screening.
5.Biopsy and angiogram of vessels and organs.
B.Buerger disease (thromboangiitis obliterans).
1.Normal CRP, ESR, immunologic panel, hypercoagulability screen, and toxicology screen.
2.Positive anticardiolipin antibodies.
3.Consider arteriogram of upper and lower extremities and aorta.
C.Primary angiitis of the central nervous system.
1.Cerebrospinal fluid (CSF): Leukocytosis with increased protein.
2.Angiograms: String of beads pattern.
3.Positive brain biopsy.
4.Clinical diagnosis made by ruling out infection, neoplasm, metabolic disorder, and cocaine use.
Differential Diagnosis
A.Other forms of vasculitis.
B.Thromboembolic events.
Evaluation and Management Plan
A.General plan.
1.Symptom management.
2.Exercise and healthy lifestyle changes.
3.Buerger disease (thromboangiitis obliterans).
a.Smoking cessation is the cornerstone of treatment.
b.Local wound care.
c.Pneumatic compression and spinal cord stimulation for pain due to ischemia.
B.Patient/family teaching points.
1.Treatment of vasculitis with corticosteroids or other immunocompromising medications causes an immunocompromised state.
2.Corticosteroids can lead to complications with diabetes, weight gain, or osteoporosis.
C.Pharmacotherapy.
1.Polyarteritis nodosa.
a.Prednisone 60 mg orally daily.
b.Methylprednisolone 1,000 mg intravenous (IV) daily for 3 days for the critically ill.
c.Immunosuppressive agents for moderate to severe disease.
2.Buerger disease (thromboangiitis obliterans).
a.Calcium channel blockers for vasospasm.
3.Primary angiitis of the central nervous system.
a.Corticosteroids and cyclophosphamide.
Follow-Up
A.Interval follow-up based on severity of disease.
Consultation/Referral
A.Refer to rheumatology.
Special/Geriatric Considerations
A.There are no specific age-related considerations in vasculitis-medium vessel.
Bibliography
Hajj-Ali, R. A., & Calabrese, L. H. (2017, July 3). Primary angiitis of the central nervous system in adults. In M. Ramirez Curtis (Ed.), Retrieved from https://www.uptodate.com/contents/primary-angiitis-of-the-central-nervous-system-in-adults
Hannon, R. A., & Porth, C. M. (2017). Porth pathophysiology: Concepts of altered health states (2nd ed.). Philadelphia, PA: Wolters Kluwer.
Merkel, P. A. (2019, March 1). Overview of and approach to the vasculitides in adults. In MRamirez Curtis (Ed.), UpToDate. Retrieved from https://www.uptodate.com/contents/overview-of-and-approach-to-the-vasculitides-in-adults
Olin, J. W. (2018, September 20). Thromboangiitis obliterans (Buerger’s disease). In K. A. Collins (Ed.), UpToDate. Retrieved from https://www.uptodate.com/contents/thromboangiitis-obliterans-buergers-disease
Papadakis, M. A., McPhee, S. J., & Rabow, M. W. (2016). Current medical diagnosis & treatment 2016. New York, NY: McGraw Hill Education.
Sundel, R. (2018, November 13). Kawasaki disease: Clinical features and diagnosis. In E. TePas (Ed.), UpToDate. Retrieved from https://www.uptodate.com/contents/kawasaki-disease-clinical-features-and-diagnosis