Write about PAN here.

A necrotising vasculitis that causes aneurysms of medium-sized vessels

• M:F = 2:1

• Sometimes associated with HBs Antigens

Signs and Symptoms:

• General features - very similar clinical picture as Churg-Strauss Syndrome, but with a negative ANCA (antineutrophil cytoplasmic antibody)

• Fevers, abdo pain, malaise, reduced weight, arthralgia

• Renal (75%) Main cause of death.

• Hypertension, haematuria, proteinuria, renal failure, intrarenal aneurysms, vasculitis

• Vascular

• Raised BP, vasculitis, erectile dysfunction

• Cardiac (80%) Second Biggest Cause of Death

• Coronary arteritis and consequent infarction

• Raised BP and HF

• Pericarditis

• With Kawasaki's (childhood PAN variant), coronary aneurysms can occur

• Pulmonary (interplay with Churg-Strauss, debatable)

• Pulmonary infiltrates

• Asthma

• CNS (70%)

• Mononeuritis multiplex

• Sensorimotor polyneuropathy

• Seizures

• Hemiplegia

• Psychoses

• GI (70%)

• Abdo pain (any viscus may infarct)

• Malabsorption due to chronic ischaemia

• Skin

• Urticaria

• Purpura

• Infarcts

• Livedo reticularis

• Nodules

• Blood

• Raised WCC

• Eosinophilia (30%)

• Anaemia

• Raised ESR

• Raised CRP

Diagnosis

Clinical features in combo with reanal or mesenteric angiography.  ANCA is classically negative

Treatment

• Treat hypertension meticulously

• Refer to experts

• High-dose prednisolone, then cyclophosphamide