Table 1. Renal and isolated ileal involvement in adult Henoch-Schönlein Purpura. Review of the literature.
|
|
Age at onset |
Sex |
Clinical manifestations |
Bioptic findings |
Treatment |
Outcome |
|||||
|
|
|
|
|
Kidney |
Ileum |
Skin |
|
Ileum |
Kidney |
||
|
Yentis et al [3] (1973) |
27 |
M |
Persistent microscopic haematuria; an episode
of frank haematuria. Abdominal pain, vomiting. Purpura. |
None. |
Grossly
inflamed and edematous terminal ileum (laparotomy). |
None. |
None. |
Remission
within 1
month |
Unchanged |
||
|
Gaskell
et al [4] (1985) |
22 |
F |
Proteinuria
(+). Abdominal
pain, anorexia, vomiting and constipation. Purpura. Arthralgias. |
None. |
Multiple
small fullthickness infarcts (laparotomy). |
None. |
Plasmapheresis (27 litres total exchange) Oral
prednisolone (60 mg/day) |
Remission
within 7
weeks |
Unknown |
||
|
Death (1992) |
57 |
F |
Microscopic
haematuria and red cell casts at onset; subsequently rapidly progressive
renal failure. Abdominal
pain, diarrhoea. Purpura. Arthralgias. |
Focal
necrotizing GN; mesangial staining
for IgA and C3. |
Vasculitis
in small to medium-sized arteries (laparotomy). |
Leukocytoclastic vasculitis; positive IgA
immunofluorescence |
i.v.
hydrocortisone (100 mg/6h
for 2 days); i.v.
methylprednisolone (1 g/day for 3
days) followed by i.v. methylprednisolone (60 mg/day) and
cyclophosphamide (50 mg/day) |
|
|
||
|
Kawasaki et al [6] (1997) |
43 |
F |
Proteinuria
(+). Abdominal
pain. Purpura. Arthralgias. |
None. |
Mucosal
and submucosal neutrophils infiltration (endoscopy). |
Leukocytoclastic
vasculitis |
i.v.
corticosteroids (50 mg/day) |
Remission
within 1
month |
Remission
within 1
month |
||
|
Present
case |
56 |
M |
Serum
creatinine: 1.7 mg/dl (GFR: 60 ml/min); proteinuria:
5,580 mg/24h; telescopic urinary sediment. Abdominal
pain, diarrhoea. Purpura. Arthralgias. |
Proliferative
diffuse necrotizing GN; loop and mesangial staining for IgA |
Leukocytoclastic vasculitis (endoscopy). |
Dermal
capillaritis; positive IgA immunofluorescence |
i.v.
methylprednisolone (500 mg/day
for 3 days) followed by oral prednisone (1 mg/kg/day); i.v. cyclophosphamide (200 mg/day for 3 days)
followed by oral cyclophosphamide (2
mg/kg/day) |
Remission
within
7
days |
Unchanged |
||
GN
= glomerulonephritis; i.v. = intravenous; GFR = glomerular filtration rate