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Vasculitis and Thrombophlebitis Clinical Presentation

  • Author: Nadia Jennifer Chiara Luca, MD; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Jun 30, 2015
 

History

As vasculitides are systemic processes, a comprehensive history and full review of systems is necessary.

Possible triggers include the following:

  • Recent infections (upper respiratory tract infections, especially streptococcal, tuberculosis [TB])
  • Vaccinations
  • New medications

Presenting features include the following:

  • Constitutional symptoms - Fever, weight loss, fatigue, malaise (These occur in 90-100% of patients with granulomatosis with polyangiitis [GPA], formerly Wegener granulomatosis) and microscopic polyangiitis [MPA].[18, 6]
  • Skin – Nodules, which may be painful, erythematous, or ulcerated; purpura; petechiae; papulopustular lesions; erythema nodosum; livedo reticularis; Raynaud phenomenon; lower extremity swelling
  • Recurrent oral and/or genital ulcerations that heal with scarring
  • Nasal/sinus – Allergic rhinitis, nasal congestion, recurrent epistaxis, sinus pain, tearing
  • Pulmonary – Chronic cough, hemoptysis, shortness of breath, stridor, wheezing, chest pain
  • Musculoskeletal – Joint pain or swelling, myalgias, calf pain
  • Renal – Hematuria, hypertension
  • GI tract – Abdominal pain, hematochezia, vomiting, nausea
  • Geritourinary – Testicular swelling and/or pain
  • Vascular – extremity claudication, blood pressure difference between limbs, pre-syncope/fainting spells
  • Neurological – Headache, seizure, focal neurological deficits (eg, stroke, cranial nerve palsy, vision loss, fine motor deficits, movement abnormalities), diffuse neurological deficits (eg, cognitive decline, poor school performance), mononeuropathy or polyneuropathy (eg, numbness, paresthesias, hand/foot drop, weakness), visual/auditory hallucinations, fluctuating/decreased level of consciousness/encephalopathy

Past medical history may include the following:

  • Recurrent sinusitis and pneumonias
  • Asthma and allergic rhinitis precede development of Churg-Strauss syndrome

Family history may include an increased risk of Kawasaki disease in siblings.

Clinical features common in Henoch-Schönlein purpura include the following:

  • Palpable purpuric rash (must be present)
  • Diffuse abdominal pain
  • Arthritis (acute, any joint) or arthralgia
  • Hematuria and/or proteinuria
  • Lower limb swelling may be seen

Clinical features common in Kawasaki disease include the following:

  • Fever, at least 5 days in duration, unresponsive to antibiotic therapy
  • Polymorphous exanthem (nonvesicular)
  • Perineal desquamation
  • Changes in peripheral extremities (erythema and/or edema)
  • Cervical lymphadenopathy, usually unilateral, larger than 1.5 cm
  • Bilateral, non-purulent, conjunctivitis
  • Changes of lips and oral cavity - Injection of oral and pharyngeal mucosa, fissured lips
  • Irritability, arthralgia/arthritis
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Physical

General

Vital sign measurements should include 4-limb blood pressure measurement to look for a difference of more than 10 mm Hg between limbs.

Head and Neck

Funduscopy and ophthalmologic assessment for conjunctivitis, uveitis, scleritis and/or retinal vasculitis, and optic neuritis is indicated. Orbital inflammation (lid edema/erythema, proptosis) may be seen in granulomatosis with polyangiitis and microscopic polyangiitis.[22]

Inspection of nasal cavity for bleeding, ulceration, and septal perforation is indicated.

Other findings may include the following:

  • Nasal bridge swelling, pain or saddle-nose deformity (See the image below.)
    Patient with Wegener granulomatosis and saddle-nosPatient with Wegener granulomatosis and saddle-nose deformity.
  • Paranasal sinus tenderness
  • Cervical lymphadenopathy
  • Oral ulcers seen in Behçet disease
  • Otitis, mastoiditis, hearing loss

Respiratory

The following may be noted:

  • Increased work of breathing, tachypnea, hypoxia
  • Decreased breath sounds
  • Stridor, wheeze
  • Blood-tinged sputum

Cardiovascular

The following may be noted:

  • Pulselessness, inequality of pulses
  • Bruits over major arteries
  • Increased capillary refill time
  • Abnormal heart sounds, including gallop rhythm, muffled heart sounds, rub

Abdominal and genitourinary

Findings may include the following:

  • Abdominal tenderness (focal or diffuse)
  • Hepatomegaly, splenomegaly
  • Blood on rectal examination
  • Genital ulcers or scars in Behçet syndrome
  • Testicular pain and/or swelling

Dermatologic

All patients with Henoch-Schönlein purpura have palpable purpuric rash, typically over buttocks and lower extremities.

Dermatologic manifestations of Kawasaki disease include polymorphous exanthem and red/swollen extremities.

Patients with polyarteritis nodosa have deep red tender subcutaneous nodules that can become ischemic and necrotic (see the images below).

Tender erythematous nodules in cutaneous polyarterTender erythematous nodules in cutaneous polyarteritis nodosa (PAN).
Nodules on sole of foot in cutaneous polyarteritisNodules on sole of foot in cutaneous polyarteritis nodosa (PAN).
Necrotic lesions of polyarteritis nodosa (PAN). Necrotic lesions of polyarteritis nodosa (PAN).

Livedo reticularis may also be seen in childhood polyarteritis nodosa.

Musculoskeletal

These findings may include the following:

  • Arthritis (joint swelling, effusions, decreased range of motion and/or stress pain)
  • Muscle tenderness

Neurologic

These findings may include the following:

  • Peripheral neuropathy, paresthesia
  • Mononeuritis multiplex
  • Cranial nerve palsies, vision loss, visual field defects
  • Altered level of consciousness
  • Focal neurological deficits, including stroke
  • Diffuse neurological deficits
  • Hallucinations, bizarre behavior
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Causes

Secondary vasculitis

Infectious causes include the following:

Systemic rheumatic disease causes include the following:

Inflammatory bowel disease is a cause.

Lemierre syndrome is an anaerobic suppurative thrombophlebitis of the internal jugular vein and is most commonly a complication of pharyngeal, dental, or mastoidal infection.

Hypocomplementemic urticarial vasculitis is also a cause.

Malignancy causes include the following:

  • Leukemia
  • Lymphoma
  • Bronchopulmonary blastoma, angioblastoma (both rare)

Hypersensitivity vasculitis (leukocytoclastic vasculitis) and drug-induced ANCA vasculitis (propylthiouracil, hydralazine) are also both noted.

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Contributor Information and Disclosures
Author

Nadia Jennifer Chiara Luca, MD Fellow in Pediatric Rheumatology, University of Toronto Faculty of Medicine, Canada

Disclosure: Nothing to disclose.

Coauthor(s)

Susanne Maria Benseler, MD Pediatric Rheumatologist, Section Chief, Alberta Children's Hospital; Associate Professor, Department of Pediatrics, University of Calgary Faculty of Medicine, Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Barry L Myones, MD Co-Chair, Task Force on Pediatric Antiphospholipid Syndrome

Barry L Myones, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, Texas Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Christine Hom, MD, to the development and writing of this article.

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Preferred sites of vascular involvement by selected vasculitides.
Patient with Wegener granulomatosis and saddle-nose deformity.
Tender erythematous nodules in cutaneous polyarteritis nodosa (PAN).
Nodules on sole of foot in cutaneous polyarteritis nodosa (PAN).
Necrotic lesions of polyarteritis nodosa (PAN).
Chest radiography in Churg-Strauss syndrome (CSS) with pulmonary infiltrates.
CT of sinuses in a patient with Wegener granulomatosis (WG) showing erosion and loss of sinus walls.
CT chest in a patient with Churg-Strauss syndrome (CSS) showing multiple nodules.
Table 1. EUVAS disease categorization of ANCA-associated vasculitis
CategoryDefinition
LocalizedUpper and/or lower respiratory tract disease without any other systemic



involvement or constitutional symptoms



Early



systemic



Any, without organ-threatening or life-threatening disease
GeneralizedRenal or other organ-threatening disease, serum creatinine >500



μmol/L (5.6 mg/dL)



SevereRenal or other vital organ failure, serum creatinine >500 μmol/L (5.6 mg/dL)
RefractoryProgressive disease unresponsive to glucocorticoids and cyclophosphamide
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