Polyarteritis Nodosa

Written by Dr. PAWAN TIWARI

Polyarteritis Nodosa(PAN) or Panarteritis nodosa or periarteritis nodosa or Kussmaul diseases or Kussmaul-Maier

Auto-immune inflammatory systemic disease of medium  or small size vessels, particular those in muscles. It’s mainly affect middle age (30-45 yr),more common in male . No association with ANCA.

Etiology:  Idiopathic, infections, reaction to medications, also associated with hepatitis B and C.

Pathogenesis: Immunecomplex deposition(e.g. HBsAg/anti-HBsAg)

PAN can involve any organs system  of our body some listed below:

1. Skin: vesicular rash, papule , petechial  purpura, livedoreticularis ,nodules under the skin.
2. Muscles and bone: joint and muscles syndrome or arthritis, arthralgia myesthenic syndrome with myalgia.
3. Peripheral nervous system: asymmetrical polyneuropathy ,crania
   
   l nerve damage ,elbow nerve damage ,etc.
4. CNS : stroke,psychosis
5. Kidney: vesicular kidney pathology with chronic kidney failure very rare with  GN.
6. Heart  and  vessels system: coronaritis with clinical signs of angina ,MI  and arterial HTN.
7. GIT:  abdominal syndrome (pancreatitis, cholecystitis, appendicitis, GIT  bleeding, liver damages with liver infarction ,etc) post prandial pain.
8. Gonad: Orchitis , epididymitis
9. Eye: conjunctivitis, uveitis, obstruction of central artery 

10.Lung(rare):lungs vasculitis, interstitial pneumonia with fibrosis and pleuritis, hemoptysis, coughing  sudden dyspnea .

 Classification of PAN:

A) Clinical classification:

1. Acute(less than 3 months)
2. Sub-acute(more than 3 months but less than 6 or 7 months)
3. Chronic(more than 7 months)

B) By degree of activity:  (by giving points)

1. 0 (absent of disease activity)
2. 1 (minimal)
3. 2 (medium)
4. 3 (sever/high)

C) Stages of PAN :

1.Primary

2.open

3.terminal

Sign and symptoms:

- Most prominently are the neuro/neuromuscular symptoms which are present in 70 percent of patients.These includes peripheral neuropathy,transient loss of vision and mononuritis multiplex.

- Post prondial pain.

- Hypertension 

- edema 

- oliguria

- uremia

- constituinal symptoms like fever weakness ,malaise fatigue,wt.loss ,loss of  apatite.

- muscles and joint ach

- peripheral neuropathy

- heart attack ,heart failure ,stroke, seizers, pericarditis.

- palpable purpura

- livedo reticularis

- mouth ulcers

- vesicular skin rash

Diagnosis:

No specific test exist for PAN. DX  mainly passed on clinical  manifestations and some lab tests.

- CBC: Increase WBC, anemia of chronic disease , Increase ESR, Elevated  Creactive protein

- Clinical UA(urine analysis): proteinuria, glucosuria, Increase relative density or may be normal.

- Microscopic for bacterial infection 

ACR (American collage Of Rheumatology) criteria 

Patients must have 3 or more of the following  10 criterion in order to dx PAN:

1.Wt.loss of 4kg for first 5 months.

2. Levido reticularis.

3.Testicular pain or tenderness.

4.Myalgia (different but not back pain) or leg weekness or tenderness.

5. Mononeuropathy or polyneuropathy(because of PNS and CNS nerves involvement)

6.Diastolic BP >90 mmHg 

7:Increase BUN creatine level  >133 mmol/l

8:Arteriogram demonstrating aneurysm or occlusion of visceral arteries.

9:Presence of HBsAg in serum

10:Biopsy of small or medium- sized artery (granulomation and mononuclear filtration of vessels). The  sural nerve is frequent location for biopsy.

Instrumental diagnosis :

• Chest X-ray
• USG( do find problem in abdominal cavity organs)
• Echo.of Heart
• Angiography
• ECG
• Fundoscopy 
• CT –scan of brain chest ( faster than MRI)
• MRI (better for soft tissue,its contraindicated in pregnant women ,child ,body part having metals,pacemaker users,to detect for heart valves,etc.)
• Spirometry
• Biopsy of kidney or lungs or affected parts .

Treatment Plan :

1st line : 

- Prednisone : 1mg/kg/day orally 2 times a day for two month after  that it’s doses should be decreased  and should be given5-15 mg per day for further 3-5 months.

- Cyclophosphamide: 1-2 mg/kg/day orally for 10-14 days and then it’s doses should be decreased. In severe PAN 4 mg/kg/dayfor 3 days ,Then 2mg/kg/day for further 7 days after that doses should  be given 25-50 mg /month.

Note: general  treatment  with these first line drugs is for approx 14 months.These two drugs are gold standard for tx of PAN.

In pts. With HBV or HCV related  PAN  ,cyclophosphamide can not be used.

As usual cyclophosphamide may be substituted with MTX or Azathioprine.

Note: prolonged used of cyclophosphamide leads to bladder cancer .

Effective therapy: Plasma exchange, lymphocytophoresis or immunoabsorption

Anti-coagulant: Heparin 5000 IU 4 times a day SC (under the skin)

NSAIDS: Ibrufen :800-1200 mg per day , Celecoxib-200 mg per day.

Angioprotectors: tribenosid,etc

Antiviral drugs :for PAN which associated HBV and HCV.

Antibiotic: cephalosporin 3rd generation, broad spectrum antibiotics  like amoxicillin.

Enzymotherapy.

Others Tx :

Symptomatic treatments..

Surgical treatment: pneumolobectomy,nephrectomy,etc.