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4th Glomerulonephritis-Workshop: IgA-Nephropathy
This workshop took place in Tübingen, Germany, in 1993. The results were published in the journal "Nieren- und Hochdruckkrankheiten", volume 23, No. 11/1994 (Dustri-Verlag Dr. Karl Feistle, München-Deisenhofen, Germany). Reprints can be ordered from the authors or the publisher directly.
(Nieren Hochdruckkrh 1994 (23) 538 - 540).
After Berger's first report on a
nosological entity of recurrent hematuria and proteinuria
together with mesangial IgA-depositions increasing information
exists in the literature to correlate the disease with further
etiological, epidemiological and prognostic characteristics. 1.5
to 35 % of patients with renal biopsy show IgA nephropathy
reaching peaks in Japan and France compared to low frequencies
in Northern America (USA + Canada). Finding of IgA deposits is
frequently related to infections of the respiratory tract.
Key words: Mesangial IgA deposits - etiological and
epidemiological factors.
(Nieren Hochdruckkrh 1994 (23)
In patients with mesangial IgA glomerulonephritis
impaired activity of suppressor-inducer T-cells, decreased
suppressor T-cell activity and a hyperactivity of T-helper cells
is found. This results in increased production of IgA and
IgA-immune-complexes. It is suspected that oral tolerance is
also disturbed in IgA-nephropathy.
The familial occurrence and deviations in allele frequencies of
certain HLA-subtypes, complement components and of
immunoglobulin genes point to a genetic predisposition for IgA
nephropathy. It is presently unknown how IgA immune complexes
result in glomerular mesangial damage.
Key words: Mesangial IgA glomerulonephritis -
Pathophysiology.
(Nieren
Hochdruckkrh 1994 (23) 548 - 550).
Clinical presentation of IgA
nephropathy may be as asymptomatic urinary abnormality or as
episodes of macroscopic hematuria with or without proteinuria.
25 % of these patients will develop renal failure within 15
years.
The clinical course depends on proteinuria (>
1 g/day bad
prognostic sign), onset of arterial hypertension, and a
preexisting moderate renal failure. Patients with recurrent
macroscopic hematuria seem to have a more favorable prognosis.
Morphological findings give further prognostic indications.
Women have a better outcome. Pregnancy does not influence the
course of the disease.
Key words: IgA nephropathy - clinical course - risk factors.
(Nieren
Hochdruckkrh 1994 (23) 551 - 555).
IgA nephropathy is
characterized by immunohistological deposits of IgA, IgG and
complement. Light microscopically, diffuse or focal
proliferative as well as sclerosing alterations are conspicuous.
IgA nephropathy differs from other types of glomerulonephritis
by its obvious classical symptoms hematuria, proteinuria,
hypertension as well as by a typical constellation of
immunological parameters. Infections of the upper respiratory
tract, inflammations of the gastrointestinal tract and allergies
are regarded as predisposing diseases. Hematuria with
demonstrable dysmorphic erythrocytes is considered as an
early symptom, but acanthocyturia does not differentiate between
this disease and other types of glomerulonephritis. Mostly,
proteinuria is below 1 g/day; unselective proteinuria is not
favorable for prognosis. Hypertension appears to be less
frequent than other types of the disease. An increase in the
IgA, subclass is regarded as pathogenetic, as an evidence of
systemic immunodysregulation. The diagnostic importance of
monomerically and polymerically bound IgA in immunecomplexes, of
IgA rheumatic factor activity, of IgG autoantibodies or of IgA
specific antibodies against surface molecules of endothelial
cells is not yet understood. Clinical and laboratory findings
are useful to classify activity, development and therapeutic
effects. Until now, the disease can not be unambiguously
diagnosed without renal biopsy.
Key words: IgA nephropathy - hematuria - proteinuria
hypertension - immunological parameters.
(Nieren Hochdruckkrh 1994 (23)
556 - 558). Clinical evidence of a "secondary" IgA nephropathy
was investigated in 26 patients (pts) with alcoholic liver
cirrhosis (LCl), 35 pts with posthepatitic LCI and compared with
results of 2 control groups (group I: 18 pts with "idiopathic"
IgA nephropathy (IgA-N); group II: 12 pts with a benign IgA
gammopathy). Results: 15 of 18 pts with IgA-N (control group I)
had clinical signs of a glomerulopathy (microhematuria,
proteinuria), only 4 of 18 IgA-N pts had an elevated serum IgA.
All 12 pts with IgA gammopathy (control group II) had an
elevated serum IgA but none of them clinical signs of a
glomerulopathy. Only 1 of 35 pts with posthepatitic LCI and 3 of
26 pts with alcoholic LCI had clinical evidence of a "secondary"
IgA nephropathy.
Conclusions: There is only a minor clinical evidence for a
"secondary" IgA-nephropathy in pts with LCI although
morphologically mesangial IgA has been described in a higher
incidence of LCI pts.
Key words: Alcoholic liver cirrhosis - posthepatitic liver
cirrhosis - IgA nephropathy - serum IgA - benign IgA gammopathy.
(Nieren Hochdruckkrh
1994 (23) 559 - 563)? IgA glomerulonephritis is the relatively
most common form of chronic glomerulonephritis. It can be
separated into a primary idiopathic and a secondary form.
"Secondary" means either coincidence or association with another
primary disease or the multiorganic manifestation of a basic
immunological pathogenesis. A multitude of primary lesions can
be accompanied or followed by IgA-glomerulonephritis. Such a
complex systemic manifestation is likely to result in a worse
prognosis compared to pure primary IgA-glomerulonephritis. The
proportion of primary to secondary IgA-glomerulonephritis is
determined in part by age (e.g. children with a high proportion
of associated Schoenlein-Henoch Purpura) and in part by the
pattern of patient selection - but last not least also by the
indication for renal biopsy which we often perform in
active disease processes. We thus found 30 (26 %) of 117
histologically proven IgA-glomerulonephritis to be secondary
forms.
Key words: Secondary IgA glomerulonephritis - systemic diseases
- "connecto-vasculitis"and "vasculo-connectivitis" -autoimmune
disease process.
(Nieren Hochdruckkrh 1994 (23) 564 - 569.
The therapy of the primary IgA
nephropathy is still a matter of controversy despite numerous
clinical trials. During the past decades, various
immunosuppressive therapy protocols were tried with different
success rates in addition to mere supportive measures such as
blood pressure control, prescribing diuretics and dietary
regimens. The main problem in evaluating the effect of any
immunosuppressive therapy for this condition is the small number
of cases in the literature. Meta-analysis was used as a tool to
answer the question whether the immunosuppressive therapy has
any valuable effect for the outcome of patients, their
proteinuria and renal function. All available data since the
first description of the disease up to 1993 were pooled.
There was a significant benefit for patients with nephrotic
range proteinuria and IgA nephropathy in order to reduce urinary
protein excretion. But there was no difference in patients with
proteinuria less than 3.5 g per day. Neither patients on
immunosuppressives nor patients on exclusive supportive care
showed a difference in their renal function by the end of the
observation period.
Key words: IgA nephropathy - glomerulonephritis therapy -
immunosuppressive agents - corticosteroids - cyclophosphamide -
chlorambucil - azathioprine - cyclosporin A meta-analysis.
(Nieren Hochdruckkrh 1994 (23) 570 -
IgA nephropathy is one of the most frequent of primary
glomerular diseases in adults. The course of the disease is
rather variable, its prognosis is generally regarded as
relatively good although 20 years after diagnosis about 20-30 %
of the patients suffer from end stage kidney disease. Besides
the question which patient needs therapy at all, the
kind of therapy is still under discussion. In the following
symptomatic therapeutic approaches are considered. Of great
importance may be a consequent antihypertensive therapy
preferably with an ACE-inhibitor. Measures to reduce
(tonsillectomy) or modify serum IgA levels or IgA-containing
immune complexes may reduce symptoms like gross hematuria, but
no consistent effect on the progression of the disease has been
observed.
Key words: Immunoglobulin A - IgA nephropathy - ACE
inhibitor - tonsillectomy.
(Nieren
Hochdruckkrh 1994 (23) 575 - 579).
Protein loading is known to
increase renal blood flow without influencing heart rate or
systemic blood pressure. In 24 patients with IgA nephropathy
glomerular filtration rate, effective renal plasma flow and
urinary albumin excretion were examined before and after an
acute protein load. An increase in GFR was only observed in
patients with a baseline GFR >
75 ml/min/ 1.73 m2. Sixteen
hypertensive patients were reevaluated after 4 weeks treatment
with either perindopril or nifedipine. Systolic and diastolic
blood pressure were lowered in these patients. Both drugs did
not affect the glomerular response to acute protein loading. We
conclude that protein loading induces glomerular hyperfiltration
in patients with IgA-nephropathy as long as GFR is above 75
ml/min. Treatment with perindopril or nifedipine in patients
with IgA glomerulopathy did not impair GFR and did not alter the
hemodynamic response to amino acid loading.
Key words: IgA glomerulopathy - arterial hypertension renal
functional reserve - perindopril - nifedipine.
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