C1q Nephropathy - A profile

Abstract

AbstractBackground C1q nephropathy is
an under diagnosed disease with varied
clinical presentation. Diagnostic criteria
of C1q nephropathy 1. Dominant or
codominat immunoflurescent staining for
c1q in kidney biopsy2. Mesangial electron
dense deposits 3. No clinical and
serological evidence of SLE.Materials
and methods 13 patients who satisfied
the above criteria were studied. Clinical
profile and laboratory parameters including
urine analysis, urine spot PCR, blood
biochemistry, serum complement and
histopathological profile were analyzed.
Creatinine clearance was estimated using
Cockgraut Gault formula. They were
followed followed up for assessment of
response to treatment.Results Among
the 13 patients 12 were female (92.3). All
(100) were hypertensive at the time of
presentation. Age ranged from 15 to 48
yrs with mean of 34 yrs. Microscopic
haematuria was found in all 13 patients (
(100) Nephrotic proteinuria was found 10
patients (77) and 4 patients (30.7) had
GFR60mlmin. Kidney biopsy revealed
Diffuse Proliferative Glomerular Nephrits
in 12 patients (92.3) one patient had
FSGS (7.7). Cellular crescents were
found in 2 patients (15.3). One patient
was lost for follow-up. 3 patients (25) improved
with ACE inhibitors and statins. 9
patients (69.2) were started on steroids
of which the 4 patients who had renal failure
received cyclophosphamide in addition
to steroids. Of the 9 patients, complete
remission was found in 2 patients
(22) , partial remission in 2 patient(22)
and no response to immunosuppressive
mediation was found in 5 patients(55.5)
( 1 patient had FSGS and 4 patients had
DPGN)Conclusion among the 13 cases
with c1q nephropathy1.All patients had
hypertension and microscopic hamaturia.
2.Nephrotic proteinuria was seen in
three fourth of the patients3.Commonest

histopathological presentation was diffuse
proliferative Glomerulonephrits.4.Half of the
patients showed poor response to oral steroids