Over the last 10 years, 17 patients (13 males and 4 females) diagnosed
as having classical polyarteritis nodosa (PAN) were treated and follo
wed up in the rheumatology clinic of our institute. The median age and
duration of symptoms at presentation were 29 years (range 13-59) and
9.5 months, respectively. Patients presented with the typical clinical
picture of classical PAN. The diagnosis was established with the help
of an aortogram (7/10), sural nerve biopsy (7/8), muscle biopsy (5/7)
and renal biopsy (3/4). For reasons not known, none of the 17 patient
s was HBsAg positive. Patients were treated with a combination of oral
prednisolone (1 mg/kg per day) for 6 weeks, which was slowly tapered
off over 6 months, and monthly intravenous cyclophosphamide pulses (15
mg/kg) for the first 6 months, followed by 3-monthly pulses for a tot
al of 2 years. Remission was achieved in 14 patients after a median of
5 months of treatment. Remission was stable for a median of 5 years o
f follow-up. Three patients did not respond well and died within 6 mon
ths of diagnosis. The causes of death in these were intracerebral haem
orrhage in one patient and gastrointestinal bleeding in two patients.
This experience is in accord with the reported literature that classic
al PAN is mostly a monophasic disease with either an excellent respons
e to the appropriate immunosuppressive therapy and a long remission or
a downhill course culminating in death. A chronic course is rare.