POLYARTERITIS NODOSA | home
clinic
History: Patients usually present with nonspecific signs and symptoms such as fever, weakness, headache, abdominal pain, weight loss, and malaise. Classic PAN may involve any organ system.
 Renal system - About 60% of PAN involves kidney causing renal failure or hypertension. Commonly, it presents with ischemic changes in the glomeruli.
 Musculoskeletal system - Commonly manifests as arthritis, arthralgia, or myalgia
 Central nervous system - Cerebral arteritis usually presents late in the course of the disease, usually second to third year of vasculitis, and may cause arterial thrombosis, intraparenchymal and subarchnoid hemorrhage. Global CNS dysfunction with encephalopathy and seizures may result from metabolic derangements secondary to multiple organ failure. Acute or subacute myelopathy with paraparesis can occur at any cord level. Myelopathy may also result from cord compression by extramedullary hematoma secondary to ruptured spinal aneurysm.
 Peripheral nervous system - Peripheral neuropathy is very common with PAN (up to 60% of patients). Vasculitic neuropathy is often asymmetric and either presents as (1) mononeuritis multiplex, (2) distal polyneuropathy, or (3) cutaneous neuropathy. It commonly takes the form of mononeuritis multiplex or of a pure motor, sensory, or mixed sensorimotor polyneuropathy.
 Gastrointestinal tract - GI symptoms constitute specific and nonspecific symptoms and signs such as abdominal pain, nausea and vomiting, bleeding, bowel infarction and perforation, cholecystitis, hepatic infarction, or pancreatic infarction.
 Skin - About 40% of patients may manifest dermatologic symptoms including rash, purpura, nodules, cutaneous infarcts, livido reticularis, and Raynaud's phenomenon.
 Cardiovascular system - Cardiac involvement is also common in PAN (up to 35%), which includes congestive heart failure, myocardial infarction, and pericarditis.
 Genitourinary system - Patients may develop pain over testicular or ovarian area.
The most common initial complaints are:
fever (85%);
abdominal pain (65%);
symptoms of peripheral neuropathy,
often a mononeuritis multiplex (50%);
weakness (45%);
and weight loss (45%).
Hypertension (60%),
edema (50%), and
oliguria and uremia (15%) may be present in the 75% of patients with renal involvement;
proteinuria and hematuria are early manifestations.
Diffuse or localized abdominal pain, nausea, vomiting, and bloody diarrhea may mistakenly suggest an acute surgical abdomen, although acute ischemia of the gallbladder or bowel may cause perforation and peritonitis.
Hemorrhage from the GI tract or into the retroperitoneal space may occur.
Precordial pain occurs in 25% of patients, but an ECG indicates coronary disease in 45%.
CNS disease produces headache (30%) and convulsions (10%).
Myalgias with areas of focal ischemic myositis and arthralgias are common; frank arthritis of large joints may occur.
Skin lesions, including palpable purpura, palpable subcutaneous nodules along the course of the affected artery, and irregular areas of necrosis, occur in a few patients.
Laboratory Findings
Leukocytosis of 20,000 to 40,000/µL (80% of patients), proteinuria (60%), and microscopic hematuria (40%) are the most frequent abnormalities. Transient or permanent eosinophilia is unusual but may occur in patients with an extended clinical course or with Churg-Strauss syndrome with pulmonary involvement or asthmatic attacks. Thrombocytosis, markedly elevated ESR, anemia caused by blood loss or renal failure, hypoalbuminemia, and elevated serum immunoglobulins occur frequently. Autoantibodies, although often encountered in other collagen vascular diseases, are rare.
Physical: Since PAN is a systemic disease careful general and neurological examinations are essential for diagnosis.
 Systemic examination
 Skin rash or nodules
 Raynaud's phenomenon
 Congestive heart failure
 Pericarditis
 Bowel infarction
 Cholecystitis
 Arthritis
 Hypertension
 Renal failure
 Neurological examination
 Peripheral neuropathy (sensorimotor)
 Encephalopathy
 Sensory deficit with cord level
 Focal weakness or hemiparesis
Other diagnostic considerations
 Wegner's granulomatosis (WG) involves both pulmonary and renal system. WG is almost exclusively associated with c-ANCA but not with perinuclear type of antineutrophil cytoplasmic antibody (p-ANCA). In PAN, both c- and p-ANCA can be found even though p-ANCA is more commonly associated with PAN.
Lab Studies:
Antineutrophil cytoplasmic antibody (ANCA) test
 No diagnostic serologic tests are available for PAN. Positive p-ANCA titers are often found; however, they are not diagnostic.
 Other tests
 Leukocytes are usually elevated with neutrophil predominance.
 Erythrocyte sedimentation rate (ESR) is almost always elevated.
 Hypergammaglobulinemia can also be found in 30% of PAN patients.
Imaging Studies:
Arteriograms
 Reveal typical microaneurysms in the small- and medium-sized arteries of the kidneys and abdominal viscera
Other Tests:
Electroencephalogram (EEG)
 EEG shows generalized slow wave activity during periods of active encephalopathy or toxic delirium.
Cerebrospinal fluid (CSF)
 CSF is often normal.
Procedures:
 Biopsy of small arteries from the abdominal viscera can be done; in conjunction with arteriograms it helps in identifying the expected vasculitis.
Histologic Findings: Microscopic polyangiitis is seen with PAN; however, it is also observed with Wegner's granulomatosis.
Prognosis
Acute or chronic untreated disease usually is fatal, often ending in failure of the heart, kidneys, or other vital organs or in GI catastrophes or ruptured aneurysm.
Without therapy, only 33% of patients survive for 1 yr; 88% die within 5 yr.
Glomerulonephritis with renal failure occasionally responds to therapy, but anuria and hypertension are ominous findings; renal failure is the cause of death in 65% of patients.
Potentially fatal nosocomial or opportunistic infections are common.
Staging: N/A
 |
TREATMENT
|
 |
Medical Care:
 Favorable outcome is expected with the combination of prednisone (1 mg/kg/day) and cyclophosphamide (2 mg/kg/day).
 Prednisone therapy alone can improve the long-term outcome significantly, but combined therapy with immunosuppressants may have superior outcome.
Surgical Care: Microcoil embolization of cerebral aneurysm
Consultations:
 Cardiology
 Gastrointestinal
 Dermatology
 Rheumatology
 Neurology
Diet: n/a
Activity: n/a
from emedicine
 |
MEDICATION
|
 |
Immunosuppression has been the standard therapy for PAN. Recent data suggest that combination of 2 or more different immunosuppressants can improve the outcome.
Drug Category: Immunosuppressants - Immunosuppression
Drug Name
|
Prednisone
|
Adult Dose
|
1 mg/kg/d
|
Pediatric Dose
|
1 mg/kg/d
|
Contraindications
|
n/a
|
Interactions
|
n/a
|
Pregnancy
|
B - Usually safe but benefits must outweigh the risks.
|
Precautions
|
n/a
|
Drug Category: Alkylating agents, antineoplastic - Immunosuppression
Drug Name
|
Cyclophosphamide (Cytoxan, Neosar, Procytox [Canada]) - It is used to treat cancer of the ovaries, breast, blood and lymph system, nerves, retinoblastoma, multiple myeloma, and mycosis fungoides.
|
Adult Dose
|
2 mg/kg/d
|
Pediatric Dose
|
Usually not recommended
|
Contraindications
|
Immunization is contraindicated during or immediately after treatment with cyclophosphamide.
|
Interactions
|
n/a
|
Pregnancy
|
X - Contraindicated in pregnancy
|
Precautions
|
Cyclophosphamide is excreted into the breast milk. Because this drug may cause serious side effects, breast-feeding is generally not recommended.
|
Drug Category: Antimetabolic agents, antineoplastic - Immunosuppression
Drug Name
|
Methotrexate (Rheumatrex, Folex, Arbitrexate, Methotrate, Mexate) - It interferes with cell growth. Methotrexate is used in the treatment of psoriasis, certain forms of cancer, and certain connective tissue diseases such as rheumatoid arthritis, lupus, polyarteritis nodosa, and scleroderma.
|
Adult Dose
|
5-7.5 mg/wk
|
Pediatric Dose
|
n/a
|
Contraindications
|
Immunization is contraindicated during or immediately after treatment with cyclophosphamide.
|
Interactions
|
Anticoagulants - increased anticoagulant effect; anticonvulsants - possible methotrexate toxicity; antigout drugs - decreased antigout effect, toxic levels of methotrexate; asparaginase - decreased methotrexate effect; clozapine - toxic effect on bone marrow; flurouracil - decreased methotrexate effect; folic acid - possible decreased methotrexate effect; isoniazid - increased risk of liver damage; leukovorin calcium - decreased methotrexate toxicity; levamisole - increased risk of bone marrow depression; NSAIDs - possible increased methotrexate; phenytoin - possible increased methotrexate toxicity; probenecid - possible methotrexate toxicity; pyrimethamine - increased toxic effect of methotrexate; salicylates - possible methotrexate toxicity; sulfadoxine, pyrimethamine - increased risk of toxicity; tetracyclines - possible methotrexate toxicity
|
Pregnancy
|
X - Contraindicated in pregnancy
|
Precautions
|
Alcohol can increase the likelihood of liver damage from methotrexate.
Since methotrexate passes into breast milk, both the manufacturer and the American Academy of Pediatrics advise against use by any woman who is breast-feeding.
|
follow up:
Further Outpatient Care:
 PAN is a chronic disease with various neurological features, which are multifocal and fluctuating. Following symptoms should be carefully monitored:
 Headache
 Uncontrolled hypertension
 Dementia
 Psychosis
 Encephalopathy
|