1. 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.

2. NON AMYLOID, FIBRILLARY AND IMMUNOTACTOID GLOMERULOPATHIES.
Presented By:
Dr. Habib-ur-Rahman
Registrar
June 2008

3. 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.

4. 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.

5. 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.

8. 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.

9. 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

10. 5% Increment

11. THANX