Polyarteritis Nodosa Clinical Presentation

Updated: Dec 03, 2018
  • Author: Dana Jacobs-Kosmin, MD, FACP; Chief Editor: Herbert S Diamond, MD  more...
  • Print
Presentation

History

Polyarteritis nodosa (PAN) is an acute multisystem disease with a relatively short prodrome (ie, weeks to months). [7] 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, [44] and depression

Next:

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.) [45]

  • CNS involvement - Although rare (≤10% of cases), encephalopathy, focal deficits, strokes, seizures, and, sometimes, brain hemorrhages can occur. [45, 46]

Cutaneous symptoms

Cutaneous symptoms in PAN include the following [7] (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 [32] :

  • 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 [47] ; an additional patient had disease limited to the soleus and gastrocnemius muscles of his calf. [48]

Previous