Polyarteritis nodosa in north India: clinical manifestations and outcomes.

Objective: There has been a significant decrease in the number of published reports of classical polyarteritis nodosa (PAN) in the post-Chapel Hill consensus conference (CHCC) nomenclature era with only two series published from Asia. We report a case series of PAN from north India.

Patients And Methods: A retrospective study of all patients diagnosed to have PAN according to American College of Rheumatology criteria/CHCC nomenclature. The details of clinical presentation, investigation findings, treatment details and outcomes were noted from the records. These findings between the hepatitis B positive and negative groups were compared.

Results: Twenty-seven patients (20 male, seven female) were diagnosed as having PAN, out of which seven (25.9%) were hepatitis B surface antigen positive. Nervous system involvement was most common with 24 patients (88.9%) having mononeuritis multiplex. Weight loss was present in 20 (74%), fever in 14 (51.9%), renal involvement in 16 (59.3%), cutaneous in nine (33.3%), peripheral gangrene in eight (29.6%), gastrointestinal (GI) involvement in eight (29.6%), testicular pain in 6/20 (30%) and cardiac involvement in four (14.8%). Twenty-three (85.2%) patients recovered, three died (11.1%) and one was lost to follow-up. Median follow-up duration was 37 (interquartile range 22.00-69.75) months. The cumulative survival was 114.16 months (95% CI: 98.27-129.95). There was no significant difference in five factor score (FFS) or revised FFS between those patients who died and those who survived (P = 0.248, 0.894, respectively). Hepatitis B-related PAN had a lower FFS compared to non-hepatitis B-related PAN (P = 0.039). No other significant differences were noted between the two groups.

Conclusion: In comparison to classic PAN in other populations, classic PAN in north India is associated with higher neurological involvement and lower GI involvement.