What are the patient and renal outcomes in W.G. and MPA?

 

CLINICAL  NEPHROLOGY

 

Revise please the abbreviation list on:

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

Q.180. What are the patient and renal outcomes in W.G. and MPA.?      

A. Renal outcome: As kidney is a frequent target organ in WG & MPA, progressive R.F. is commonly observed. ESRD occur in 10-26 % . Severe R.I. does NOT preclude induction of remission or clinically significant improvement in R. function, as :

Poor R. outcome:🠞[More severe R. dysfunction at presentation, lack of resp. to initial ttt, R. relapses, age >65 y., & severe fibrosis, e.g. interstitial fibrosis & G. sclerosis on initial R. biopsy ]. By comparison, response to im/m. & improved R. function over time can be obs. among ptns é predominant active R. lesion at  presentation.

Q.181. How can CRRT affect patient and renal outcomes in W.G. & M.P.?

A. CRRT:  Little is known about optimal ttt & outcome in WG/MPA requiring maintenance DX and/or R.Tx.There’re two issues need to be addressed in ptns on maintenance DX: whether to continue im/m. therapy; frequency & ttt of relapse. Some ptns progress to ESRD & need maint D.X. despite initial resp. to im/m.. and, in some cases, maint therapy. Further management varies é clinical setting:

If: NO evidence of active R. dis. (i.e. No hematuria or dysmorphic RBCs é urinery sedment, which must be distinguished for isomorphic (normomorphic) hematuria due to Cph. bladder inj.) & NO active extrarenal disease, it’s unclear how much benefit provided by maint therapy to relapse since the rate of relapse is dcrease in DX. Ptns without active disease & progress to DX. while on maint thpy discontinue maint. if ttt =3-6 m., ttt should: aza., as methx. shd NOT be given in DX. or have mod./sev. CKD.

If NO evidence of active R. disease but have active extraenal dis. despite induction é Cph, ptn shd be ttted for Cph-resistant vasculitis. Similar consideration apply to pers-istent dysm. hematuria, with or without extrarenal manif.. Purpose of keeping thpy é no extraR. manif. is: control of R. vasculitis might hs enough recovery of R. function to stop DX. Ttt ch. DX. + active WG/ MPA = same as those who don’t require maintenance DX., é duration of therapy & regimen being based upon ptn response & whether or not relapse occurs. Given increase risk of B.M. suppression é oral Cph: use cautiously é careful monitoring.. Dose adj. in DX. for oral Cph is 50% of usual dose post session & 75% of us. dose in CAPD. Methx shd NOT be used for either remission induction or maint therapy in DX.. Importance of infection:  Occurence é increase rate & ass. é mortality in WG/MPA who req. ch. H.DX. Infection was almost twice as freq. é ch. H.DX. who ttted é im/m. therapy compared to ptns on ch. DX. not ttted é im/m. thpy. Sites including:[Resp. & ur. tracts, skin, DX. access sites, peritonitis & bacteremia of unknown origin].

Q.182. What are the patient and renal outcomes in W.G. & M.P. after renal transplantation?

A. There’s limited experience é R.T.x. in WG/MPA. Tx. shd be delayed for at least 6 m. fr. time of initial presentation or most recent relapse. Pres. of +ve ANCA at time of Tx. does not predict recurrence of G.N. in Tx. organ. So, persistence of isolated ANCA positivity is NOT a C.I. to R.Tx.. It’s likely that im/m. used for prevention of Rj. & Prevention of dis. Flares in ANCA-associated vasculitis. Paucity of data exists about long-term prognosis.

 

Q.183. What is the triad of Wegener’s syndrome? How much common is this disease?

A. “Triad”of W.G.:   👌

1.    Upper & Lower respiratory.

2.    Glomerular dis.

3.    Systemic “necrotizing” vasculitis.

The Pauci-immune RBGN incl.: Wegener’s & M.P. are the most common form of “crescentic” G.N. in all ages esp. in elderly.

Q.184. What is the classic histology of Wegener’s?

A. Classic histology: “Focal Segmental Necrotizing Crescentic G.N”. The G. Crescent = [Epithelioid histocytes + Giant cells].   E/M.: Gabs = Rents in the G.B.M. through which. Fibrin & Leukocytes extravasate. No typical electron dense deposit (Pauci-immune) but rather fluffy lucent material, subendothelial & intravascular.

Q.185. What is the main difference between (Classic) P.A.N. & M.P.?

A. P.A.N. is {a systemic necrotizing medium-sized vasculitis, affecting msc. arteries at   ➵ branching points  + “aneurysm” formation }. But M.P. a necrotizing small vessel vasculitis

affecting small a., v. & capill. of kidneys, lungs, dermis, but  without  aneurysm formation.

Q.186. Describe the pathology in these patients ?

A. {Focal segmental necrotizing G.N. + Crescent formation}.

Q.187. What is the presentation of these patients? What are the most common co-morbid diseases?

A. {Constitutional Sm.s: (fever - malaise - wt. loss.)- Nausea –Vomiting.-abdominal pain- bowel infarct -  G.I. bleeding.} .

- H.B.V. & Hairy cell Leukemia are The most common co-morbid diseases.

 

Q.188. Which drugs can induce ANCA positivity?

A. Propyl thiouracil (antithyroid) & Amphetamine abuse🠞ANCA +ve polyangiitis.

Q.189. What is Churg-Strauss syndrome “Triad”?  👌

A. Churg-Strauss syndrome (allergic granulomatosis & angiitis) is an arteritis é eosinophilic granuloma é arterial wall, hs the foll. Triad:  👌

      Asthma.

      Peripheral eosinophilia.

      Systemic Vasculitis.

Q.190. How to manage?

A. Dg.x.: C.P., chest x-ray: [patchy, nodular or diffuse infiltrations é pl. effusion].

Serology: P-ANCA +ve, é positivity correlate well é renal & pulm. dis.

                                 ttt.: { Steroids- Aza – Cph - infliximab (TNF. blocker.) }.

Q.191. Mention the tetrad of Henoch-Schönlein purpura? How to classify?

A. Classic tetrad includes:      ✌ ✌

1)   Palpable purpura.

2)   Abd. pain.

3)   Arthritis, ankle, knee.

4)    Hematuria.   (Mesangial proliferation G.N., typical Ig A.).

- Henoch-Schönlein purpura classification:

                     I.        Minimal changes. 

                   II.        Mesangial proliferation.

                 III.        Mesangial proliferation + < 50 % crescents.      

                  IV.        Mesangial  proliferation + > 50 % crescents.

Q.192. What are the poor prognostic criteria in H.S.P.?

A. Old age, severe Nephrosis, heavy proteinuria, biopsy criteria: [severe interstitial fibrosis- IgA deposit-fibrinoid necrosis].

Q.193. What is “Nutcracker” syndrome ?    

“Nutcracker” syndrome 

A. It’s a rare cause of hematuria occur due to: Compression of the “Left R.V.” between the “Abdominal aorta” & the proximal “Superior mesenteric” artery.

Q194. What are the renal diseases associated with rheumatoid arthritis?  

A. Two ✌ main categories:

  I. Direct complications of the dis.:

1)   M.N.

2)   MesPGN (± Ig A or Ig M deposits).

3)   Diffuse proliferative G.N.: Necrotizing & Crescentic G.N. (Rhoid vasculitis).

4)   Amyloidosis. (AA type).

  II. Complications of “rheumatoid therapy”:

(1) Gold: ➪ M.N.- MCD- ATN.

(2) Penicillamine: ➪M.N.-MCD- Crescentic G.N.

(3) NSAID: ➪ Ac TIN with MCD- MCD without TIN- ATN.

(4) Csp: ➪ Ch. vasculopathy – TIN- FSGS.

(5) Aza/6 mercaptopurine: ➪ Acute Interstitial Nephritis.

(6) Pamidronate: ➪ FSGS.

Q195. What are the collagen vascular (Rheumatic) diseases associated with glomerular lesions?

A. Collagen vascular (Rheumatic) diseases associated glomerulopathy:

(1) SLE: see Q.143.

(2) Rhoid arthritis: see Q.210.

(3) Rheumatic fever:➪rarely, MesPGN. PSGN & Ac. rhc. fever never co-exist (different strains).

(4) Ankylosig spondylitis & Reiter’s synd.:➪ MesPGN, IgA , AA amyloidosis.

(5) Mixed connective tissue disease:➪MN- MesPGN.(10-50%).

(6) Scleroderma:➪ MesPGN, sev. AKI (Scleroderma renal crises: Sev. hyper-reninemic H.T. encephalopathy, systolic/diastolic CHF, AKI), ttt.: ACEI, R.Tx.

(7) Relapsing polychondritis:➪ Crescenntic  G.N., MesPGN, M.N.

(8) Dermatomyositis/Polymyositis:➪ MesPGN., IgA, rarely: AKI (myoglobulinuria.).

(9) Systemic or renal limited polyangiitis:➪ Crescenntic  G.N.