Transcript Causes, clinical features, pathogenesis, fibrillary glomerulopathy

This lecture was conducted during the Nephrology Unit Grand
Ground by Registrar under Nephrology Division under the
supervision and administration of Prof. Jamal Al Wakeel, Head
of Nephrology Unit, Department of Medicine and Dr.
Abdulkareem Al Suwaida, Chairman of Department of
Medicine. Nephrology Division is not responsible for the
content of the presentation for it is intended for learning and
/or education purpose only.
NON AMYLOID, FIBRILLARY AND
IMMUNOTACTOID
GLOMERULOPATHIES.
Presented By:
Dr. Habib-ur-Rahman
Registrar
June 2008
Causes
• Haematological malignancies, most common.
• (CLL,NHL,MGUS.)
• Non hematologic malignancies (5%) (including lung ,colon and
•
•
•
•
•
•
renal cell carcinomas)
lympho-proliferative disease (2%)
myelo-proliferative disease (2%)
vasculitis (3%)
hepatitis or cirrhosis (5%).
Other autoimmune or inflammatory conditions included thyroiditis,
rheumatoid arthritis, temporal arteritis, gout, bronchiectasis, and
intravenous drug abuse.
HIV.
Clinical Features.
• Immunotactoid Glomerulopathy.
• 1.NS with Heamaturia.
• 2.Hypertension.
• 3.RPGN.
• 4.Pulmonary Hemorrhage
• Fibrillary Glomerulopathy.
• 1.NS with microhematuria.
• 2.Hypertension
• 3.Microtubular inclusions in leukemic lymphocytes.
PATHOGENESIS
A. Immunotactoid Glomerulopathy.
1.Composed of microtubules >30nm.
2.Organized in Parallel arrays
3.Composed of Monocolnal IgG.
B. Fibrillary Glomerulopathy.
1.Composed of Fibrils.(12-22nm)
2.Random distribution
3.Composed of Polyclonal IgG.
Renal Pathology
The Osler Institute Review Course
Fibrillary Glomerulopathy
Light Microscopy
•Mesangial hypercellularity
•Mesangial expansion
Immunofluorescence
Microscopy
•Linear staining staining with
IgG, C3, kappa and lambda
Renal Pathology
The Osler Institute Review Course
Fibrillary Glomerulopathy
Electron Microscopy
• Randomly distributed
fibrils
• Diameter – 20 to 30 nm
Treatment.
• Fibrillary Glomerulopathy
responds poorly to Corticosteroids
and Cytotoxic Drugs.
Incidence of ESRD is 50 %.
• Immunotactoid Glomerulopathy.
Corticosteroids and /or Chemotherapy were
associated with partial or complete remission in
most of cases.
• Trnsplantation.
•
Recurrence of disease in most of cases.
Conclusion
• FGN is an idiopathic glomerular disease characterized by randomly
arranged Congo red–negative fibrils of mean 20 nm diameter.
Glomerular deposits are predominantly composed of polyclonal IgG
and C3, with strong evidence of monotypic or oligotypic IgG
subclass restriction.
• IT is defined by glomerular deposition of generally larger
microtubules that are composed of monoclonal IgG in the majority
of cases.
• Although renal presentation of FGN and IT are similar, patients with
IT are more likely to have dysproteinemia, hypocomplementemia,
and occult cryoglobulinemia.
• Based on these important clinical and immunopathologic
distinctions and their implications for patient management, we
believe the differentiation of FGN from IT is useful and well justified
5% Increment
•THANX