GLOMERULAR DISEASE

Define “Crescent”?

 

 GLOMERULAR DISEASES

Revise please the abbreviation list on:

https://draft.blogger.com/u/0/blog/post/edit/8610857019469578230/4564412989605988372

Q.78. Define “Crescent”? 

 

A. Severe glomerular cpll. wall injuryNon specific response: fibrin leak into Bowman’s space ⮞ Parietal cell proliferation & mononuclear phagocyte migration into G. tuft fr. circulation. Large crescents ⮞ compress G. cpll. ⮞ 🠟 filtration. Although crescent can resolve, Chemotactic signals recruit fibroblasts ⮞ Crescent & whole G. Scarring  ⮞ ESRD.

Q.79. Define RBGN?

A. Evidence of G. dis. [proteinuria/hematuria, dysmorphic RBCs  & RBCs Casts] +Rapid decline of R. function é days or weeks = RBGN. If unttt ⮞R.F. The Hallmark of RBGN⮞Crescent in R. biopsy. Incidence: only 2-4 % of all G.N.s

Q.80 Classify primary(Iry) RBGN?

A. Iry RBGN: 👌     

      Type I. “Anti-GBM” A.B. dis. (Goodpasture dis.).

      Type II. Granular “immune- complex” associated.

      Type III. “Pauci-immune” G.N.

Q.81. Classify secondary (disease-associated) RBGN?

A. II.ry RBGN: 

         Superimposed on Iry G.N.

           Goodpasture’s Syndrom:  

 

            Carcinoma.

            Medications:

      Allopurinol.

      Penicillamine.

            Postinfectious: 

      Visceral abscess.

      Poststreptococcal G.N.

            Vasculitis: (a) Small vessel:

                                         i.    M.P.A.

                                        ii.    S.L.E.

                                       iii.    Cryoglobulinemia.

                                       iv.    Wegener’s Granulomatosis.

                                        v.    Churg-Strauss syndrome:     

  

                                       vi.    Henöch-Schönlein purpura.

                              (b) Medium vessel: P.A.N.

 

Q.82. How to narrow the spectrum of the D.D. of the aforementioned causes?

A. see:

1)   Complement: C3 & C4⮞ Normal, unless: underlying SLE is present.

2)   ANA: is a useful screen for: SLE & connective tissue dis..

3)   Anti-G.B.M. A.B. ⮞ Goodpasture dis.

4)   ANCA ⮞ for small vessel disease dgx., alth. dis. may be kidney-limited. Normal kidney function vs. Azotemia + (hematuria/proteinuria).

Q.83. What renal condition associated with linear binding of I.g. to GBM?

A. Linear binding of I.g. to the GBM may be specific or non-sp.:

    I. Specific binding:

1)   Goodpasture’s syndrome.

2)   Alport’s syndrome after R.Tx.  

   II. Non-sp. binding:  

1)   D.M.

2)   Cadaver kid.

3)   Light chain dis.

4)   Fibrillary glomerulopathy.

5)   SLE. (Possibly sp. but not pathogenic).

 

Q.84. What is anti-G.B.M. A.B. dis.?

A.Circulating A.B. to Ag site é type 4 collagen in GBM⮞ Diffuse crescentic GN.

Q.85.What is the difference between Goodpasture's syndrome & G.p. dis.?

A. Goodpasture's syndrome has the “triad” of :

1)   Pulm. hge.

2)   A.B. to G.B.M.                                          

3)   Proliferative Crescentic G.N.

However, some use the term Goodpasture's synd. for the clinical constellation of  [G.N. + pulm. hge, regardless of the underlying pathogenesis ]. The term Good-pasture's dis. is often reserved for those ptn.s é [G.N., pulm. hge.+ anti- GBM A.B.].    

Q.86. What are the early “predictors” of pulmonary hge in this syndrome?

A.   Predictors of pulmonary hge:   ✌ ✌ 

            1.    Unexplained Anemia.

2.    Hemosiderin-laden macrophage.

3.    High quality chest-X-ray criteria.

4.    Increased alveolar/arterial O2 gradient

Q.87. How to manage?

A. Dg.X.:   C.P. * R. Biopsy: I.F.: Intense diffuse Linear staining of G. B.M.

                                               E/M.: No Electron dense deposits.

                                  * ttt.: [Pph. + Csp.+ Pulse steroids ].

 

Q.88.What are the major glomerular lesions associated with neoplastic desiseas?

 A. Major glomerular lesions associated with neoplastic desiseas:

1.    Lung carcinoma⮞ IgA Np.

2.    Leukemia & lymphoma ⮞ FSGS.

3.    Cancer colon, breast, stomach & lung ⮞ M.N.

4.    Ch. lymphocytic leukemia, lymphoma(é HCV) ⮞ MPGN.

5.    Cancer prostate, pancreatic cancer, hodgkin’s l.oma, mesothelioma ⮞ MCD.

6.    Lung carcinoma ⮞Crescentic G.N./Systemic vascultis.

7.    Renal cell carcinoma⮞ AA systemic amyloidosis.

8.    M. Myeloma, Waldenstrom’s macroglobulinemia ⮞ AL amyloidosis.

9.    Lymphoma & myeloma ⮞Light chain Np.

10.  Lymphoma⮞Fibrillary (immunotactoid) G.N.

11.  Gastric cancer, mucin producing cacer  ⮞ HUS.

12. Ch. lymphocytic leukemia (é HCV) ⮞ Cryoglobulinemic G.N.

Q.89. How can the histopathologic variants of FSGS predict the clinical course of the disease?

A. Histopathologic variants of FSGS & the clinical course:

1)   G.”Tip” lesion: Swelling,vaculation& proliferation of visceral epithelial cells (Podocytes) & sclerosis of é G. segment closest to proximal tubules⮞ Benign course + Good response to steroid thpy.   😃

2)   Collapsing variant: focal or global G. collapse & sclerosis é visceral epithetlial cell swelling⮞ Poor prognosis, common in African Americans & HIV ptns.  😌

3)   Severe tubulointerstitial disease ⮞ Poor long-term renal survival.     😌

Q.90.What are the factors associated with steroid resistance in treating FSGS?

A.“Steroid resistance”:     ✌ ✌

1)   Advanced R.I.

2)   Severe tubulointerstitial dis.

3)   Massive proteinuria > 10 g./d.

4)   Familial variants.

Q.91. How can you diagnose primary membranous nephropathy (M.N.)?

A.Up to 30% of M.N. in adults & 80 % in children ⮞ 2^ndry causes are found.

- 75% of 2^ndry M.N., due to ⮞ [SLE, HBV, Malignancies & medications].

Q.92. Enumerate 2^ndry causes of M.N.?

A. Secondery  causes of M.N.:

1)   Infections:  

1.    HBV.                 

2.    HCV.                

3.    Malaria.            

4.    Filariasis.  

5.    Helicobacter pylori.

6.    Castleman’s dis.

7.    Leprosy.        

8.    Hydatid dis.

9.    Schistomiasis.

10. Syphilis.

11. Scabies.

2)   Autoimmune:    

1.    S.L.E.                       

2.    Sarcoidosis.                

3.    Sjögren’s dis. 

4.    Crohn’s dis.

5.    Grave’s dis.   

6.    Graft vs. host dis.

7.    Guillain-Barre synd.

8.    Rhoid arthritis.  

9.    Dermatomyositis.       

10. Dermatitis Herbitiformis.

11. Ankylosing spondylitis

12. Anticardiolipin A.B. synd.

13. panniculitis.

14. Mysthenia Gravis.

15. Mixed connective tissue

16. Hashimoto’s thyroiditis.     

17. Bullous pemphigus

18. Urticarial vasculitis.

19. Weber-Christian dis.

3)   Malignancies:

1.    Carcinoma (lung-colon-breast- stmach-esophagus).

2.    Leukemia/Lymphoma (non-Hodgkin’s).

3.    Melanoma.

4)   Medications: [Gold-Mercury- Captopril- D-penicillamie- Probenicid- NSAID].

5)   Genetics: [Sickle cell dis.-Fanconi synd.- Sclerosing cholangitis- D.M.].

6)   Others:[Hydrocarbon- Kimura’s dis.-De Novo in R. allograft.].

Q.93.What laboratory test should exclude M.N. diagnosis?

A. Low Complement should exclude M.N. EXCEPT:       

1)               Membranous lupus.

2)               HBV-induced M.N.

 

Q.94. How common is Ig A nephropathy (IgA Np.)?

A. IgANp ⮞ the most common form of G.N. worldwide. Most cases are subclinical, but prevalence is highest in western Pacific Rim & low in U.S. It’s uncommon in African American.  { ♂ : ♀ = 2 : 1} .

Q.95. How much beneficial is the measurement of IgA plasma level?

A. Only 50 % of cases é IgA Np ⮞  elevated pl. level of IgA. However, measuring  IgA level is ⮞ Neither sensitive, nor specific to be used in Dgx. of IgA Np.  👎

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