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Polyarteritis Nodosa Clinical Presentation

  • Author: Dana Jacobs-Kosmin, MD, FACP; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Jan 12, 2016
 

History

Polyarteritis nodosa (PAN) is an acute multisystem disease with a relatively short prodrome (ie, weeks to months).[5] Delays in diagnosis are not uncommon. The spectrum of disease ranges from single-organ involvement to fulminant polyvisceral failure. Pertinent and common historical features of PAN include the following:

Constitutional and musculoskeletal symptoms

Constitutional and musculoskeletal symptoms of PAN include the following:

  • Fever
  • Malaise
  • Fatigue
  • Anorexia and weight loss
  • Myalgia
  • Arthralgia in large joints or, less commonly, arthritis

Central nervous system symptoms

Transient symptoms of cerebral ischemia, including typical spells of transient monocular blindness, are the most common presenting CNS deficits of PAN. Cerebral arteritis usually presents late in the course of the disease, usually in the second to third year of the vasculitis. Cerebral arteritis may cause arterial thrombosis with cerebral ischemia or intraparenchymal or subarachnoid hemorrhage.

Global CNS dysfunction with encephalopathy and seizures results from metabolic derangements secondary to multiple organ failure. Acute or subacute myelopathy with paraparesis can occur at any cord level. Myelopathy may result, although rarely, from cord compression by an extramedullary hematoma secondary to a ruptured spinal aneurysm. Although CNS lesions usually occur 2-3 years after the onset of PAN, earlier CNS involvement has been reported.

Peripheral nervous system symptoms

Peripheral neuropathy develops in as many as 60% of patients. Vasculitic neuropathy is often asymmetrical and presents as (1) mononeuritis multiplex, (2) distal polyneuropathy, or (3) cutaneous neuropathy. It can take the form of a pure motor, pure sensory, or mixed sensorimotor polyneuropathy.

Cutaneous symptoms

Dermatologic symptoms are very common in PAN, and about 40% of patients manifest with skin lesions including rash, purpura, gangrene, nodules, cutaneous infarcts, livido reticularis, and Raynaud phenomenon. Skin involvement, which can be painful, occurs most frequently on the legs.

Gastrointestinal symptoms

GI involvement usually presents as nonspecific symptoms and signs such as abdominal pain (which may be postprandial) and nausea and vomiting, with or without obvious GI bleeding. Rare and more serious complications of PAN include bowel infarction and perforation, cholecystitis, hepatic infarction, or pancreatic infarction.

Renal symptoms

About 60% of patients with PAN have renal involvement. Flank pain may be present. Ischemic changes in the glomeruli and renal artery vasculitis can cause renal failure, hypertension, or both. A small percentage of patients may require dialysis.

Additional symptoms

Less common symptoms reported in PAN include the following:

  • Genitourinary - Patients may develop pain over the testicular or ovarian area. In rare cases, testicular infarction may occur; testicular pain is usually unilateral
  • Cardiac - Chest pain, dyspnea, palpitations, pericarditis, myocardial infarction, and congestive heart failure; cardiac disease affects 35% of patients with PAN, but most affected patients are asymptomatic
  • Ophthalmologic - Blurred vision
  • Neuropsychiatric - Headache, psychosis, [39] and depression
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Physical Examination

Because polyarteritis nodosa (PAN) is a systemic disease, a complete examination is essential for diagnosis.

The emergence of multiple mononeuropathies in persons with PAN is an important clue to an underlying arteritis. Evidence of organ or extremity ischemia, including hypertension and renal insufficiency (renovascular disease), are further clues to the diagnosis. Lung involvement is rare and its presence should prompt a search for an alternative to PAN as the diagnosis.

Constitutional and musculoskeletal symptoms

Fever may be observed; Raynaud phenomenon may occur. A nondeforming, asymmetrical arthritis, usually involving the larger joints of the lower extremities, has been reported in PAN.

Neurologic symptoms

Neurologic symptoms in PAN include the following:

  • Sensory and/or motor neuropathies - When these occur, they are usually asymmetrical
  • Mononeuritis multiplex (multiple mononeuropathy) - This is the successive ischemia or infarction of "named nerves" (eg, ulnar, radial, peroneal, sural). Although nerve involvement is initially asymmetrical, the development of additional nerve lesions can cause the clinical picture to resemble symmetrical polyneuropathy. (A history of asymmetry at the onset and by electrodiagnostic studies can be helpful in this case.) [40]
  • CNS involvement - Although rare (≤10% of cases), encephalopathy, focal deficits, strokes, seizures, and, sometimes, brain hemorrhages can occur. [40, 41]

Cutaneous symptoms

Cutaneous symptoms in PAN include the following[5] (see the images below):

  • Livedo reticularis that does not blanch with active pressure
  • Ulcerations - Especially on the lower extremities, near the malleoli and on the calf
  • Digital ischemia - May be accompanied by splinter hemorrhages and, sometimes, gangrene
  • Nodules - Usually on the lower extremities (like ulcers); nodules are the least common skin manifestation of PAN
    Tender, hyperpigmented, firm subcutaneous nodules Tender, hyperpigmented, firm subcutaneous nodules with a background of livedo reticularis common in cutaneous polyarteritis nodosa (PAN).
    Tender erythematous nodules with central "punched Tender erythematous nodules with central "punched out" ulcerations common in cutaneous polyarteritis nodosa (PAN).
    Livedo reticularis in polyarteritis nodosa (PAN). Livedo reticularis in polyarteritis nodosa (PAN).

Gastrointestinal symptoms

Gastrointestinal symptoms include the following[29] :

  • Tender abdomen with or without rigidity, guarding, or diminished bowel sounds
  • GI bleeding
  • Bowel infarction
  • Cholecystitis

Renal symptoms

Renal symptoms include the following:

  • Hypertension
  • Costophrenic tenderness
  • Retroperitoneal or intraperitoneal hemorrhage
  • Renal failure

Cardiac symptoms

Cardiac symptoms include the following:

  • Hypertension
  • Tachycardia out of proportion to fever
  • Pericardial friction rub
  • Arrhythmias
  • Congestive heart failure

Ophthalmologic symptoms

Ophthalmologic symptoms of PAN include the following:

  • Retinal vasculitis
  • Retinal detachment
  • Cotton-wool spots

Additional symptoms

The patient may experience testicular tenderness. Psychiatric symptoms, specifically psychosis and depression, may occur.

Unusual presentations

PAN has been reported to present in the muscles alone. One patient had features suggesting polymyositis[42] ; an additional patient had disease limited to the soleus and gastrocnemius muscles of his calf.[43]

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Contributor Information and Disclosures
Author

Dana Jacobs-Kosmin, MD, FACP Attending Physician, Department of Medicine, Division of Rheumatology, Einstein Medical Center; Clinical Assistant Professor of Medicine, Jefferson Medical College of Thomas Jefferson University

Dana Jacobs-Kosmin, MD, FACP is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

J Mark Jackson, MD Clinical Professor of Medicine/Dermatology, Division of Dermatology, University of Louisville School of Medicine

J Mark Jackson, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Kentucky Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

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.

Acknowledgements

Mahendra Agraharkar, MD, MBBS, FACP, FASN, Clinical Associate Professor of Medicine, Baylor College of Medicine; President and CEO, Space City Associates of Nephrology

Mahendra Agraharkar, MD, MBBS, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Nephrology, and National Kidney Foundation

Disclosure: South Shore DaVita Dialysis Center Ownership interest

Bruce A Baethge, MD, Faculty, Texas A&M Medical School

Bruce A Baethge, MD is a member of the following medical societies: Alpha Omega Alpha; American College of Physicians; American College of Rheumatology and Arthritis Foundation

Disclosure: Nothing to disclose.

Kanwarpreet Baweja, MD, Fellow in Nephrology, Division of Renal Diseases and Hypertension, University of Texas Health Science Center

Kanwarpreet Baweja, MD is a member of the following medical societies: American Medical Association, American Society of Nephrology, Medical Council of India, and National Kidney Foundation

Disclosure: Nothing to disclose.

Steve Chung, MD, Director, Clinical Epilepsy Research, Assistant Professor of Clinical Neurology, Residency Director, Department of Neurology, Barrow Neurological Institute

Steven Chung, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Medical Association, California Medical Assocation, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

B Mark Keegan, MD, FRCPC, Assistant Professor of Neurology, College of Medicine, Mayo Clinic; Master's Faculty, Mayo Graduate School; Consultant, Department of Neurology, Mayo Clinic, Rochester

B Mark Keegan, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Minnesota Medical Association

Disclosure: Novartis Consulting fee; Bionest Consulting fee

Howard S Kirshner, MD, Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center

Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, National Stroke Association, Phi Beta Kappa, and Tennessee Medical Association

Disclosure: Nothing to disclose.

Carlos J Lozada, MD Director of Rheumatology Fellowship Program, Professor, Department of Medicine, Division of Rheumatology and Immunology, University of Miami, Leonard M Miller School of Medicine

Carlos J Lozada, MD is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Pfizer Honoraria Speaking and teaching; Amgen Honoraria Speaking and teaching

Christopher Luzzio, MD, Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison School of Medicine and Public Health

Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Vasanta P Weiss, MD, Consulting Staff, Department of Nephrology, University of Virginia Medical Center

Disclosure: Nothing to disclose.

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Nonspecific, firm, tender subcutaneous nodules without livedo reticularis and/or systemic involvement may be the first sign of polyarteritis nodosa (PAN).
Tender, hyperpigmented, firm subcutaneous nodules with a background of livedo reticularis common in cutaneous polyarteritis nodosa (PAN).
Tender erythematous nodules with central "punched out" ulcerations common in cutaneous polyarteritis nodosa (PAN).
Polyarteritis nodosa (PAN) is characterized by fibrinoid necrosis of the arterial wall with a leukocytic infiltrate. In this slide, a large, pale occlusion of a muscular artery can be seen. Within this collagenous tissue is a leukocytic infiltrate, which is the hallmark of PAN. Courtesy of Urbana Atlas of Pathology.
Livedo reticularis in polyarteritis nodosa (PAN).
 
 
 
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