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.Two main types.
1.Behçet disease.
2.Essential cryoglobulinemia.
Incidence
A.More common in patients of Asian, Turkish, and Middle Eastern descent.
B.Prevalence similar in men and women.
C.Commonly afflicts patients ages 20 to 40 years old.
D.Disease is more severe in young male patients from Middle East or Far Eastern Asia.
Pathogenesis
A.Vasculitis can be a primary pathology or can be secondary to another underlying disease process (Table 14.1).
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.
1.Behçet disease.
a.Affects both arteries and veins of all sizes.
2.Essential cryoglobulinemia.
a.Cold-precipitated, immune-complex mediated.
b.Occurs in chronic underlying infection (hepatitis C most commonly), connective tissue disease, lymphoproliferative disorders.
Predisposing Factors
A.First degree relative with Behçet disease increases the risk for the disease.
B.Essential cryoglobulinemia is associated with chronic hepatitis C infection.
Subjective Data
A.Common complaints/symptoms.
1.Constitutional symptoms are common with all size vessel vasculitis and include fever, weight loss, malaise, and arthralgias/arthritis.
2.Behçet disease.
a.Aphthous lesions of mouth and genitals.
b.Tender, erythematous popular lesions which ulcerate.
c.Knee and ankle arthritis.
d.Posterior uveitis, hypopyon.
e.Neurological conditions including sterile meningitis, cranial nerve palsies, seizures, encephalitis, mental status changes, and spinal cord lesions.
f.Hypercoagulable states.
3.Essential cryoglobulinemia.
a.Palpable purpura.
b.Peripheral neuropathy.
c.Abdominal pain.
d.Digital gangrene.
e.Pulmonary disease.
f.Glomerulonephritis.
B.Common/typical scenario.
1.Essential cryoglobulinemia.
a.Purpura.
b.Weakness.
c.Arthralgia.
2.Behçet disease.
a.Recurrent, painful ulcers of mouth and genitals.
b.Follicular rash.
c.Uveitis.
d.Sterile pustules at needlestick sites.
e.Arthritis.
C.Family and social history.
1.Essential cryoglobulinemia.
a.Sexual history.
b.Intravenous (IV) drug use.
c.Family history of Behçet disease.
D.Review of systems.
1.Essential cryoglobulinemia.
a.Abdominal pain.
b.Sensitivity to cold.
c.Skin changes.
d.Paresthesias.
e.Weakness.
2.Behçet disease.
a.Mucosal ulcers.
b.Skin nodules.
c.Skin rashes.
d.Joint pain.
e.Eye pain.
f.Photophobia.
g.Eye redness.
h.Visual changes.
i.Headache.
j.Weakness.
k.Mental status changes.
l.Chest pain.
m.Dyspnea.
Physical Examination
A.Behçet disease.
1.Aphthous ulcers of mouth and genitals.
2.Follicular rash.
3.Erythema nodosum-like lesions.
4.Hypopyon.
B.Essential cryoglobulinemia.
1.Palpable purpura.
2.Peripheral sensorimotor deficits.
3.Raynaud’s phenomenon.
Diagnostic Tests
A.Behçet disease.
1.Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP).
2.No definitive tests.
3.Positive pathergy test: At least a 2 mm papule will develop 24 to 48 hours after needle insertion to the skin.
B.Essential cryoglobulinemia.
1.Elevated liver enzymes.
2.Positive cryoglobulins.
3.Low C4 level.
Differential Diagnosis
A.Hepatitis B.
B.Hepatitis C.
C.HIV.
D.Connective tissue disease.
E.Lymphoproliferative disorders.
F.Other types of vasculitis.
Evaluation and Management Plan
A.General plan.
1.Based on cause and severity of the vasculitis.
2.Alleviation of symptoms.
B.Patient/family teaching points.
1.Regular follow-up is necessary to monitor condition.
2.Patients should also be educated on side effects of medications used for treatment.
C.Pharmacotherapy.
1.Behçet disease.
a.Colchicine 0.6 mg 1 to 3 times per day and thalidomide 100 mg daily.
b.Apremilast for oral ulcers.
c.Corticosteroids or azathioprine for severe disease.
d.Infliximab, cyclosporine, or cyclophosphamide for ocular and neurological disease.
2.Essential cryoglobulinemia.
a.Corticosteroids and rituximab or cyclophosphamide for 2 to 4 months.
b.Hepatitis C treatment.
Follow-Up
A.Regular follow-up determined based on severity and cause of vasculitis.
Consultation/Referral
A.Based on cause and course of disease.
B.Consider:
1.Ophthalmology.
2.Dermatology.
3.Infectious disease.
Special/Geriatric Considerations
A.Progression of these diseases is unpredictable and can affect basically all body systems.
B.Treatment is symptomatic alleviation.
Bibliography
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 M. Ramirez Curtis (Ed.), UpToDate. Retrieved from https://www.uptodate.com/contents/overview-of-and-approach-to-the-vasculitides-in-adults/print
Papadakis, M. A., McPhee, S. J., & Rabow, M. W. (2016). Current medical diagnosis & treatment 2016. New York, NY: McGraw Hill Education.
Smith, E. L., & Yazici, Y. (2018, November 13). Clinical manifestations and diagnosis of Behçet syndrome. In M. Ramirez Curtis (Ed.), UpToDate. Retrieved from https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-behcets-syndrome