Recurrence of Henoch-Schonlein purpura nephritis after 6.5 years of remission- an unusual clinical occurrence Vignesh Pandiarajan*, Deepti Suri*, Anju Gupta*, Amit Rawat*, Ritambhra Nada**, Ranjana W Minz #, Biman Saikia #, Surjit Singh* *Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India **Department of Histopathology, PGIMER, Chandigarh, India # Department of Immunopathology, PGIMER, Chandigarh, India A 6 years old boy presented with palpable purpura over lower limbs, periumblical abdominal pain, nephrotic range proteinuria and glomerular hematuria Skin biopsy showed leucocytoclastic vasculitis and renal biopsy revealed increased mesangial cellularity, endocapillary proliferation with IgA staining and cellular crescents in <50% glomeruli [Fig. 1] Received 5 daily pulses of intravenous methylprednisolone (30 mg/kg/day), followed by maintenance oral prednisolone (in gradually tapering doses) and azathioprine (at 2 mg/kg/day), which were continued for 24 and 36 months, respectively Abdomen pain and rashes subsided within a week after steroid therapy Complete remission of nephritis was attained after 11 months He remained in remission for the next 6.5 years At 14 years, he had a relapse with skin rashes, severe abdominal pain and hematuria Abdomen ultrasonogram and serum amylase levels were normal Received 5 doses of pulse IV methylprednisolone for his severe gastrointestinal symptoms Renal biopsy revealed focal necrotizing glomerulonephritis with mesangial IgA deposits Went on to develop nephrotic range proteinuria and rapid onset renal failure, which was managed with single intravenous cyclophosphamide pulse 500mg/m 2 and five cycles of plasma exchange Introduction Conclusion Late recurrences of HSP after several years of remission are extremely uncommon We speculate that long term follow up of children with HSP nephritis may be needed along with periodic urinalysis to identify recurrences Late recurrences of HSP after several years of remission are extremely uncommon We speculate that long term follow up of children with HSP nephritis may be needed along with periodic urinalysis to identify recurrences Henoch-Schonlein purpura (HSP), a common childhood systemic vasculitic disorder, is characterized by leucocytoclastic vasculitis involving predominantly small vessels Recurrence rate of HSP is about 7%- mostly occurs 2 to 5 months after initial diagnosis [1] Late occurrences are very unusual Henoch-Schonlein purpura (HSP), a common childhood systemic vasculitic disorder, is characterized by leucocytoclastic vasculitis involving predominantly small vessels Recurrence rate of HSP is about 7%- mostly occurs 2 to 5 months after initial diagnosis [1] Late occurrences are very unusual Clinical Profile Figures References 1. Jauhola O, Ronkainen J, Koskimies O, et al. Renal manifestations of Henoch– Schönlein purpura in a 6-month prospective study of 223 children. Arch Dis Child 2010;95:877– Deng F, Lu L, Zhang Q, et al. Henoch–Schönlein purpura in childhood: treatment and prognosis- Analysis of 425 cases over a 5-year period. Clin Rheumatol 2010;29:369– Byun JW, Song HJ, Kim L, et al. Predictive Factors of Relapse in Adult With Henoch-Schönlein Purpura. Am J Dermatopathol 2012;34:139– Fujinaga S, Ohtomo Y, Murakami H, et al. Recurrence of Henoch–Schoenlein purpura nephritis after long-term remission in a 15-year-old girl. Pediatr Nephrol 2006;21:1215– Jauhola O, Ronkainen J, Koskimies O, et al. Renal manifestations of Henoch– Schönlein purpura in a 6-month prospective study of 223 children. Arch Dis Child 2010;95:877– Deng F, Lu L, Zhang Q, et al. Henoch–Schönlein purpura in childhood: treatment and prognosis- Analysis of 425 cases over a 5-year period. Clin Rheumatol 2010;29:369– Byun JW, Song HJ, Kim L, et al. Predictive Factors of Relapse in Adult With Henoch-Schönlein Purpura. Am J Dermatopathol 2012;34:139– Fujinaga S, Ohtomo Y, Murakami H, et al. Recurrence of Henoch–Schoenlein purpura nephritis after long-term remission in a 15-year-old girl. Pediatr Nephrol 2006;21:1215–16. Discussion Possibility of progression to nephritis in HSP ranges from 5 to 15%. Risk factors are male sex, older age onset of disease, severe abdominal pain and recurrence of HSP [1,2]. Risk factors of HSP nephritis recurrence- older age of disease onset, persistent rash, abdominal pain, hematuria, absence of IgM deposits in vessel walls [3]. Index child had presence of IgM deposits in skin biopsy, but not in renal histology. To the best of our knowledge, recurrence of HSP nephritis after having been in sustained remission for as long as 6.5 years, has not been previously reported. HSP nephritis relapse previously reported after 6 years of remission in a 15 years old girl by Fujinaga et al [4]. Possibility of progression to nephritis in HSP ranges from 5 to 15%. Risk factors are male sex, older age onset of disease, severe abdominal pain and recurrence of HSP [1,2]. Risk factors of HSP nephritis recurrence- older age of disease onset, persistent rash, abdominal pain, hematuria, absence of IgM deposits in vessel walls [3]. Index child had presence of IgM deposits in skin biopsy, but not in renal histology. To the best of our knowledge, recurrence of HSP nephritis after having been in sustained remission for as long as 6.5 years, has not been previously reported. HSP nephritis relapse previously reported after 6 years of remission in a 15 years old girl by Fujinaga et al [4]. Figure 1. A) Lower limb examination showing multiple purpuric rashes in the calf region B) Renal biopsy immuno-fluorescence study showing IgA deposits predominantly in mesangium AB