Polyarteritis Nodosa

 

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Original Editors - Josh Bixler from Bellarmine University's Pathophysiology of Complex Patient Problems project.

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Definition/Description

Polyarteritis nodosa (PAN) is a serious ideopathic vascular disease that commonly affects both small and medium-sized arteries throughout the body. It falls under the category of primary systemic vasculitis, and with the inflammatory nature of the disease, arteries become swollen and blood flow is diminished. The inflammation, which affects the entire arterial wall, typically manifests where the arteries branch and ultimately the affected vessel tissues become necrotic. As the outer and inner layers of the artery swell, blood clots can form and potentially damage various organs and tissues in the body such as the liver, kidneys, heart, GI tract, testes, and muscles.

Prevalence

• Development and diagnosis of the disease seems to occur between the ages of 40 and 60.
• While it affects adults more so than children, it can present at any age.
• Twice as likely to occur in men than women.
• Can affect every ethnicity and race.
• Rare disease, but estimates of frequency in the US are around 77/1,000,000.
• Internationally, reports have listed frequency in south Sweden at 1.6/1,000,000; 4.6/1,000,000 in England; 30.7/1,000,000 in Paris, France.

Characteristics/Clinical Presentation

Polyarteritis nodosa is clinically similiar to many diseases such as hepatitis B and C infections, Churg-Strauss syndrome, Kawaski's disease, hypersensitivity angitis, as well as Cogan's syndrome. The speed at which the disease affects an individual often varies. Within months, it may initially present with mild symptoms that rapidly progress to fatal symptoms, or it can develop into a chronic state that incapacitates the individual. With the exception of the lungs, polyarteritis nodosa has the ability to affect many organs and organ systems at the same time by damaging the arteries that supply blood flow. The heart, intestines, liver, and kidney arteries are prevalently damaged. Listed below are some of the most common symptoms associated with this disease, affecting the skin, joints, brain, nerves, heart, digestive tract, liver, and kidneys:

• Rashes with raised patches along arteries, discoloration of fingers or toes, blotches that appear purple
• Joint pain and inflammation, muscle aches and pain
• Fever, headaches, strokes, seizures
• Weakness, numbness, tingling, or hand or foot paralysis
• Angina and myocardial infarctions
• Peritonitis (infection of the abdomen), nausea, vomiting, bloody diarrhea, intestinal tears, and rapid weight loss
• Liver damage and failure
• Hypertension, edema, decreased output of urine, urine with high levels of protein
  

Associated Co-morbidities

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Medications

Typically corticosteroids and drugs that suppress the immune system are prescribed. One such steroid is prednisone, which allows the affected individual some pain relief and can help stop the disease from causing more damage. Also, to further address the inflammation, cyclophosphamide is often prescribed in combination with the prednisone. If the cause of polyarteritis nodosa is related to a hepatitis infection, and the inflammation has been limited, anti-viral medication along with plasmapheresis is used to combat the infection.

Diagnostic Tests/Lab Tests/Lab Values

Present symptoms
Blood/urine work

Arteriogram

Electromyography

CBC

ESR or CRP

Biopsy of affected tissues

Immunoglobulin levels

X-rays

Etiology/Causes

The cause of polyarteriris nodosa remains unknown, but there does appear to be a link to drug reactions, viral infections, and the body's immune system. Adverse drug reactions to iodide or pencillin, or indiviudals with active hepatitis B or C appear to be at a higher risk for developing this disease. In fact, for approximately every five individuals affected by polyarteritis nodosa, one of those people has active hepatitis B.

Systemic Involvement

Due to its involvement with the arterial system, PAN has the ability to affect many systems in the body, notably the nervous, integumentary, renal, and GI system. Listed below are the systems most commonly affected and what is typically seen:

Nervous System:

• Peripheral neuropathy (50-70% of the time) with the individual experiencing numbness, tingling, or burning sensations in the extremities
• CNS lesions

Integumentary System: (most commonly seen affecting the legs)

• Purpura
• Livedo reticularis
• Ulcers
• Nodules
• Gangrene

Renal System:

• Flank pain
• Hypertension
• Decreased kidney function (dialysis may be needed in some cases)
• Protein in urine

Gastrointestinal System:

• GI bleeding
• Abdominal pain
• Nausea/vomiting
• Constipation
• Melena
• Hematochezia
• Possible hemmorrhage or perforation (rare)

Musculoskeletal System:

• Arthralgia
• Myalgia
• Arthritis (less common)

Cardiac System:

• Chest pain
• Tachycardia
• Pericarditis
• Myocarditis
• Arrhythmias
• Dyspnea
• Possible myocardial infarctions or congestive heart failure

Ophthalmologic:

• Blurred vision
• Scleritis

Genitourinary System:

• Testicular pain (unilateral)
• Testicular infarction

Neuropsychiatric:

• Headache
• Depression
• Psychosis

Pulmonary System:

• Involvment of the lungs is very rare

Medical Management (current best evidence)

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Physical Therapy Management (current best evidence)

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Alternative/Holistic Management (current best evidence)

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Differential Diagnosis

• Hepatitis B and C infections
• Takayasu disease
  
• Horton disease
  
• Churg-Strauss syndrome
  
• Wegener granulomatosis
  
• Microscopic polyangiitis
• Cogan's syndrome
• Kawaski disease
• Hypersensitivity angitis

Case Reports/ Case Studies

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Resources

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Recent Related Research (from Pubmed)

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References

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