Which type of therapy may be allowed for pregnant lupus?

CLINICAL NEPHROLOGY

Revise please the abbreviation list on:

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

 

Q.158. Which type of therapy may be allowed for pregnant lupus?

A. {Steroid thpy. + Aza} , can be safely used é relative safety.

Q.159. What prognostic features denoting good response?

A. Reversal of :{1. Inflammatory response (see criteria of activity above).                      2. Im/m. deposits.    3. Glomerular segmental scarring.  4. Interstitial fibrosis.} ..occ. within 6 m. denotes a favorable prognosis.  ü

160. What is the most common R. les. in mixed connective tissue disease ?  How to manage?

A.  M.N. is the most comm. les. é overlap of (S.L.E., Scleroderma & polymyositis).

- The commonest test for Dg.x.  ENA test (Extractable nuclear Ag.).  إِينَا   * Therapy. include:   [Steroids - C.C.B.- ACE - & i.v. Prostacycline for P.H. ].

    Q.161. Can amyloidosis be presented without proteinuria?

A.  Vascular amyloidosis cn be presented é little or No proteinuria.

Q.162. Which type of G. dis. primarily ass. é “fibrillary deposits”?

1.           Amyloidosis.

2.           Fibrillary G.N.

3.           Immunotactoid glomerulopathy… Other rare G. dis. incl.:

4.           Fibronectin glomerulopathy &

5.           Collagenofibrotic glomerulopathy.

Q.163. What is main histopathological difference between Amyloid fibrils & that of fibrillary G.N. & Immunotactoid G.N.?   

Ultrastructural features of fibrils of amyloid (A), fibrillary glomerulonephritis (B), and microtubules of immunotactoid glomerulopathy (C). The amyloid fibrils are randomly arranged & nonbranching. In fibrillary G.N., the fibrils are morphologically indistinguishable fr. those of amyloid but thicker. Immunotactoid glomerulopathy is ch.ch. by microtubules that are hollow when viewed on end.

A..Whereas all amyloid fibrils are Congo-red +ve, all other forms of fibrillary deposition dis. are Congo-red -ve. Furthermore, while amyloid fibrils = 8-12 nm in size, fibrillary fibrils = 16-24 nm non-branched, in random & immunotactoid fibrils = 30-50 nm hollow microtubules arranged in a parallel stacks.

Q. 164. What is Waldenström Syndrome?      A. A syndrome é which an abnormal circulating monoclonal Ig M protein + B. cell lymphoproliferative disorderinvolve the lymphocytes.

* C.P.: [fatigue- Wt. loss- hd.- bleeding P.N.- visual disturbance- HSM.] = Picture of Hyperviscosity syndrome.

Q.165. How can Waldenström Syndrome affect the kidney? How to treat?

A. Neoplastic lymphoplasmocyte cell invasion to the kidney Ü Prot. + micro-scopic hematuria.

- IgM Large intrarenal protein thrombi.          - ttt. :

v  {Pph. + melphalan + Steroids.}.

v  “Rituximab” & “B.M. Tx. ”shd be considered in sp. cases.

Q. 166.   What are the histopathological types of FSGS?

   A. (1) N.O.S. (Not otherwise specified).

        (2) Tip lesions.  

        (3) Cellular. -   

        (4) Peri-hiler.   

  

        (5) Collapsing.   

Q.167.  Enumerate 2ry causes of FSGS?

1)   General: [HIV.- Drug abuse- Interferon- Pamidronate].

2)   Genetic: [Podocin abnormalities/a-actin].

3)   Glomerulopathy: [Morbid obesity- Cong. cyanotic heart dis.- Hypoxic pulm. dis.].

4)   Reduced “Renal mass”:

                                                                           I.        Single Kidney.

                                                                         II.        Nephrectomy.

                                                                       III.        Reflux Nephropathy.

Q.168. What characterizes “Membranous Lupus”?

B   Three criteria: 

      C1q. st. é I.F.

      Mesangial dense deposits (E/M.).

      Ig. & complement (I.F.) é tub. B.M.

 

Q169. What is the difference in term definition between Goodpasture’s syndrome, Goodpasture’s dis., Anti-GBM dis. and Alport’s syndrome post-Tx anti-GBM dis.?

A. Term definition & “pathogenesis”:

Ø  Goodpasture’s synd.: [RBGN + Alveolar hge] due to several causes.]..

Ø  Goodpasture’s dis.: [Disease ass. é auto-A.B. sp. for a3(IV) NCL, due to auto-immunity to a3(IV) NCL.].

Ø  Anti-GBM dis.:[Dis. ass. é A.B. sp. for ANY component of GBM.], due mostly to: [Goodpasture’s synd. & Alport’s synd. post-Tx anti-GBM dis.].

Ø  Alport’s syndrome post-Tx anti-GBM dis.: [G.N. ass. é anti-GBM A.B. dev. after R.Tx.  in ptn. é Alport’s synd., due to immunity to foreign collagen IV chains not exprerssed in Alport’s synd. ptns, us. a3 or a5(IV)NCL.].