Scandinavian Journal of Urology and Nephrology, 2008; 42: 178
180

CASE REPORT

Mycophenolate mofetil treatment of crescentic Henoch Schonlein

nephritis with IgA depositions

FATIH DEDE1, BIRGUL ONEC1, DENIZ AYLI1, IPEK ISIK GONUL2 & KURSAD ONEC1
1

Department of Nephrology, Ankara Numune Training and Research Hospital, Ankara, Turkey, and 2Department of
Pathology, Gazi University Faculty of Medicine, Ankara, Turkey

Abstract
Mycophenolate mofetil (MMF) is considered to be a promising therapeutic agent in primary glomerulonephritis but there
are no data on the use of MMF in Henoch
Scho
nlein nephritis (HSN). Herein we report the first adult crescentic HSN
patient in whom long-term complete remission was achieved after MMF therapy.

Key Words: Crescentic glomerulonephritis, Henoch
Schonlein nephritis, Henoch
Schonlein purpura, mycophenolate mofetil,
nephrotic syndrome

Introduction
Henoch
Schonlein purpura (HSP) is a systemic
vasculitis which is mostly seen in children. It involves
small vessels, with the deposition of immune complexes containing IgA [1,2]. Renal involvement is
common in HSP and its severity is related to the
long-term prognosis of the patient [2]. Mycophenolate mofetil (MMF), an immunosuppressive agent
used in transplantation, is suggested to be a promising therapeutic agent in many autoimmune diseases,
in lupus nephritis [3] and in IgA nephropathy which
is a form of primary glomerulonephritis [4
6]. To
our knowledge are no data on the use of MMF
in Henoch
Schonlein nephritis (HSN). We report an
adult patient who had crescentic HSN with
IgA depositions who was successfully treated with
MMF.
Case report
A 21-year-old female patient was admitted to
hospital 2 weeks after an upper respiratory tract
infection with a bilateral purpuric rash, edema and
polyarthralgia on both arms and legs. The patient
was hospitalized in the internal medicine department

with a diagnosis of HSP for 10 days and discharged

after the purpuric rash began to disappear following
oral prednisolone therapy. Four days after discharge
she was admitted again with purpura which had
spread to the trunk, increased pitting edema, hematochezia, hematuria and bilateral arthritis of the
wrist, ankles, elbows and distal interphalangeal joints
of hands. On physical examination, blood pressure
was 170/90 mmHg, pulse rate 92/min, body temperature 36.58C and pretibial edema was present.
Examinations of the other systems proved normal.
Laboratory findings revealed: urea, 54 mg/dl (normal range 10
50 mg/dl); serum creatinine, 1.45 mg/
dl (normal range 0.6
1.3 mg/dl); total protein, 50 g/l
(normal range 64
83 g/l); albumin, 24 g/l (normal
range 35
55 g/l); triglycerides, 212 mg/dl (normal
range 50
160 mg/dl); and total cholesterol, 205 mg/
dl (normal range 80
200 mg/dl). The erythrocyte
sedimentation rate was 37 mm/h and a whole blood
count revealed hypochromic microcytic anemia (hemoglobin 10.1 g/dl). Other hematological and biochemical parameters were within normal limits.
Proteinuria (500 mg/dl), microscopic hematuria
and granular casts were observed on urinalysis.
Serum C3, C4, IgA, IgG and IgM levels were
normal. Twenty-four hour excretion of urinary

Correspondence: Fatih Dede, Ankara Numune Egitim ve Arastirma Hastanesi, Nefroloji Klinigi, Samanpazari, Ankara, Turkey. Tel: 90 312 508 4552. Fax:
90 312 310 4616. E-mail: fatded@yahoo.com

(Received 20 February 2007; accepted 7 July 2007)

ISSN 0036-5599 print/ISSN 1651-2065 online # 2008 Taylor & Francis
DOI: 10.1080/00365590701571514

MMF in Henoch
Schonlein nephritis

protein was 3760 mg and creatinine clearance was
63 ml/min. Antinuclear antibodies and p- and c-antineutrophil cytoplasmic antibodies were not detected.
Abdominal ultrasonography was performed and
both kidneys were observed to be of normal size.
Based on these results, HSP and nephrotic syndrome
were suspected and a renal biopsy was performed.
Crescent formation was present in 5/11 of the
glomeruli that were examined. Fibrinoid necrosis,
endocapillary proliferation and focal tubulointerstitial mononuclear cell infiltration were present in the
remaining glomeruli. On immunofluorescence examination, mesangial accumulation of IgA (),
IgM () and C3 () was established. IgG and C4
were negative (Figure 1). These findings indicated
IgA glomerulonephritis secondary to HSP. We
decided to treat the HSN with methylprednisolone
1 mg/kg/day and MMF 1000 mg twice per day, in
addition to ramipril and pentoxifylline. The oral
methylprednisolone dose was reduced to 0.5 mg/kg/
day 2 months after its initiation and after 6 months it
was given on alternate days at a dose of 0.5 mg/kg.
MMF, ramipril and pentoxifylline were continued at
the same doses. After 12 months of treatment,
creatinine decreased to 0.64 mg/dl, proteinuria decreased to 204 mg/24 h, creatinine clearance increased to 94 ml/min and the patient underwent a
second renal biopsy. This second biopsy showed
minimal mesangial cell proliferation and none of
the seven glomeruli were crescentic (Figure 2). IgA
deposition was shown to have a focal segmental
capillary pattern and the specimen was diagnosed
as pure mesangial proliferative HSN class II-A. By
reducing the doses, oral corticosteroid was stopped
after 18 months and MMF was stopped after 24
months of therapy. Creatinine, creatinine clearance
and proteinuria were 0.77 mg/dl, 108 ml/min and

Figure 1. Low-power view of the first kidney biopsy showing five

glomeruli, two of which were affected by cellular crescents.
Hematoxylin
eosin staining; original magnification40.

179

Figure 2. The second kidney biopsy shows milder glomerular

changes characterized by minimal mesangial cell proliferation
without crescent formation. Periodic acid
Schiff staining; original
magnification40.

139 mg/24 h, respectively at the end of the therapy.

The patient experienced complete remission for 18
months.
Discussion
HSP is a systemic leukocytoclastic vasculitis which is
characterized by skin, joint and gastrointestinal
manifestations that may occur successively [1,2].
Renal involvement occurs in 37% of children but is
more frequent in adults, affecting 71.7% of patients
[1]. Among cases of glomerulonephritis, HSN is
only responsible for 0.6
2% of adult nephropathies
[2]. The renal manifestations of HSP are highly
variable, and include isolated microscopic haematuria, permanent proteinuria, acute nephritic syndrome and nephrotic syndrome. Two basic findings
are mesangial cell proliferation and the formation of
epithelial crescents [1]. Although data concerning
predictive factors for renal failure in adults are more
controversial than those in children, initial altered
renal function, age 50 years, high blood pressure,
initial proteinuria exceeding 1.5 g/24 h and high
histological stage appear to be associated with a
poor prognosis [1,2].
Mycophenolic acid (MPA), which is released from
MMF by esterase activity, is an inhibitor of inosine
5?-monophosphate dehydrogenase, a key enzyme for
de novo purine synthesis and for the glycosylation of
adhesion molecules in T and B lymphocytes [4
7].
MPA selectively inhibits the proliferation of T and B
lymphocytes, the production of antibodies, the generation of cytotoxic T cells and the recruitment of
leucocytes to sites of inflammation [7]. Although no
significant benefits of MMF in IgA nephropathy were
reported in some small trials [7,8], it was suggested in

180

F. Dede et al.

other trials and in some case reports that MMF can be

effective in reducing proteinuria and restoring renal
function in high-risk IgA nephropathy patients, without any serious side-effects [4
6,9]. Although HSN is
very similar to IgA nephropathy, and histologically
indistinguishable from it, there have been no reports
of MMF treatment in HSN. Because our patients
clinical and histological findings indicated a poor
prognosis, we initially planned cyclophosphamide or
azathioprine therapy combined with corticosteroids.
When the possible long-term side-effects of this
protocol, such as infertility, bone marrow suppression
and carcinoma, were explained to the patient, she
refused the therapy. Promising reports [4
6,9] of
successful MMF therapy in primary glomerulonephritis, systemic vasculitis and IgA nephropathy
encouraged us to try the drug. Owing to the fewer
side-effects associated with MMF, we treated our
patient (with her informed consent) with 2 g/day
MMF in addition to oral corticosteroid. The therapy
was well tolerated and no side-effects occurred; the
patient had achieved complete remission at the end of
the therapy.
We conclude that MMF and a corticosteroid can
be used safely and successfully to treat HSN;
however, prospective studies are necessary to prove
the efficacy of the drug.

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