Polyarteritis Nodosa, (Updated) A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions

This book describes Polyarteritis Nodosa (updated), Diagnosis and Treatment and Related DiseasesPolyarteritis nodosa is a serious blood vessel disease in which small and medium-sized arteries become inflamed and damaged. PAN was separated into 2 sub-types according to the size of vessel involved in adults:1. Classical PAN - medium-sized vessels2. Microscopic polyangiitis (MPA) - small vesselsA less severe form called cutaneous polyarteritis nodosa (CPAN) has also been reportedCausesPolyarteritis nodosa is a disorder that involves arteries, the blood vessels that transport oxygen-rich blood to organs and tissues.
The cause is not known but it happens when certain immune cells attack the involved arteries. More adults than children obtain this disease. The tissues that are supplied by the involved arteries do not get the oxygen and nourishment they require, and become injured. People with active hepatitis B and C may have this disorder. SymptomsSymptoms and signs are due to the inflammation and ischemia of the involved organs.
Any organ may be involved with the exception of the lungs. Symptoms are produced by injury to involved organs, often the skin, heart, kidneys and nervous system.1. Abdominal pain2. Decreased appetite3. Fatigue4. Fever5. Joint aches6. Muscle aches7. Unintentional weight loss8. Weakness9. Peripheral neuropathy10. Damage to the nervous system may produce strokes or seizures.11. Renal involvement may be in the glomeruli or renal vasculatureDiagnosis:1.
Histological evidence of vasculitis2. Positive serology for ANCA3. Specific investigations strongly indicative of vasculitis and granuloma-E.g. angiography or skin biopsy4. Eosinophilia (?10%)Investigations1. Hepatitis B surface antigen is positive in 30%.2. The p-ANCA test is normally positive, but not pathognomonic.3. ESR is increased or C-reactive protein (CRP).4. FBC reveals leukocytosis with raised neutrophils.5.
Hypergammaglobulinemia happens in 30%.6. Arteriography reveals microaneurysms in the small-sized and medium-sized arteries of the kidneys and abdominal viscera7. Tissue biopsyComplicationsWithout treatment, hypertension-induced glomerulonephritis is a source of great morbidity and mortality. TreatmentIf there is no presence of hepatitis and the symptoms are fairly mild, the doctor normally will start the treatment with a steroid drug, such as prednisone, a powerful medicine that suppresses the immune system and decreases inflammation.
If there is more severe symptoms affecting the heart, nerves or kidneys, if the patient does not improve with initial treatment or if the patient has relapses after initial treatment, prednisone may be combined with an adjunct immunosuppressant medication such as:1. Cyclophosphamide (Cytoxan)2. Azathioprine (Imuran), 3. Methotrexate (Rheumatrex), 4. Mycophenolate mofetil (Cellcept) or5. Rituximab (Rituxan).
Full treatment normally requires at least one year. If polyarteritis nodosa is linked to hepatitis B or hepatitis C, the patient may be treated with antiviral medicines alone to fight the liver infection. Antiviral treatment may be added to prednisone to control arterial inflammationOccasionally, this is accompanied by plasmapheresisPlasmapheresis is a procedure in which plasma is separated from the blood, cleaned of abnormal antibodies and then returned to the blood.
Updated treatment:1. Case reports have good response to treatment with tumor necrosis factor inhibitors, such as infliximab and etanercept.2. The interleukin-6 antagonist tocilizumab has been given successfully in refractory cases.3. Effective use of the anti-CD20 agent rituximab in an adult with refractory PAN has been doneTABLE OF CONTENTIntroductionChapter 1 Polyarteritis Nodosa...