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GLOMERULAR DISEASES

How can you distinguish glomerular from extraglomerular hematuria?

 

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GLOMERULAR DISEASES


Q.148. How can you distinguish glomerular from extraglomerular hematuria?

A.   Glomerular vs. extraglomerular hematuria:  

 

Extraglomerular” 

Glomerular”

Color (macroscopic):

Red or pink.    

Red, smoky brown, or "Coca-Cola“. 

Clots

May be present.                      

Absent.

Proteinuria

usually absent.               

May be present

RBCs morphology

Normal.               

Dysmorphic

RBCs casts.

Absent .                        

May be present.

Q. 149.What is the Hallmark of S.L.E. ?  

- Auto-A.B. production of: A.N.A. & Anti-DNA, incr. Anti-DNA. titre correlate well é clinical activity.

- Anti-S.m. A.B. (25% of cases): V. specific to L. nephritis & greatly ass. é C.N.S. , R. disease., more cutaneous mnf. &, cardiopulmonary mnf. & Worse prognosis.

Q.150. What are the renal manifestations of SLE?   👓

A. Renal Manifestation of SLE:

1)   Proteinuria: 100 %, N.S.: (45-65%).

2)   Hematuria: 

*    Microhematuria: 80 %.

*      RBCs casts: 10 %.

*    Macrohematuria 1 %.

3)   Cellular casts: (30%).

4)   Reduced renal perfusion: (40-80%):

                                    🠞  RBGN: 10-20%.

                                    🠞AKI: 1%

5)   Hypertension: (15-50%).

6)   Hyperkalemia: 15 %.

      Tubular abnormalities: (a.Sm.tc.)(60-80%).

Q.151. Mention the major criteria for diagnosis?


 

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-  { 4 of Eleven criteria are necessary for Dg.x. of S.L.E. } :  

1)   Malar rash.    

2)   Photosensitivity. 

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3)   Discoid Lupus.       

4)   Dermal mamifestation.

5)   Oral/nasal ulcers.  

6)   C.N.S. mamifestation.

7)   Serositis (Pleural/Pericardial).

8)    Renal mnf. (Persistent Prot. ½ g./d.).

9)   Cytopenias.

10) Arthritis (Non-defomitive).     

11)Im/m. markers: [ANA-Anti-DNA- Anti-Sm.].

Q.152. What is Full House  in S.L.E. ? What’s its significance?


A. Full House=After booker hands =Christmas Tree in S.L.E. = Pres. of   [Ig.: G+A+M+ C3+ C1q.] altogether in I.F. study of R. biopsy, of a lupus ptn..

-  Full House & C1q staining  Strongly suggestive of “Lupus Nephritis”.

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IF-anti IgG: The immunofluorescence profile is “full house”, meaning that IgG, IgA, IgM C3 & C1q are usually present. This image displays mesangial and granular loop staining for IgG.

IF-anti-IgA: Both mesangium & capillary loops are stained. Note the intensity is less than with anti-IgG.

IF-anti-IgM: Again both mesangium & capillary loop staining is observed, however there is slightly more within the mesangium. Note the intensity is also less than that seen with anti-IgG.

IF-anti-CHYPERLINK "http://pathology.osu.edu/residents/StudyGuides/EM/SLEnephropathy/SLEnephritis.htm"1HYPERLINK "http://pathology.osu.edu/residents/StudyGuides/EM/SLEnephropathy/SLEnephritis.htm"q: Staining here’s v. similar to tht sn é anti-IgG. Often the intensity rivals tht of anti-IgG.

IF-anti-C3: C3 is the most common complement observed in SLE, with C1q almost as common.

Q.153. Give the new classification of S.L.E.?

i.Minimal mesangial (N. in L.M.). 

ii.Mesangial proliferation.     

iii.Focal (<50%) proliferative.        

iv.Diffuse (>50%) proliferative.

v.Membranous Lupus.

vi.Advanced Sclerosis.

Q. 154.  How can you monitor “Lupus Activity”? What are the signs & symptoms of Lupus Flare?

 A. - “Parameters of activity”:

(1)         Active urinary sediment (Dysmorphic RBCs. & RBCs casts).            

(2)         Hypocomplementemia .

(3)         Anti-DNA titer. 

(4)         High E.S.R.

(5)         High C.R.P.

(6)         Circulating im/m. complexes. 

(7)         Cytokine & IL. Level.

(8)         Systemic mnf. (fever, fatigue, asthenia).

(9)         Activity features in biopsy:  [fibrinoid necrosis.- Leukocytic infiltration- Wireloop deposits. - Hyaline thrombi- H/E. Bodies].

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*** Sn.s & Sm.s of Lupus Flare: Ptn shd be aware of one or more of these Sm.s:

v  Persistent fatigue out of proportion é expected.

v  Persistent weakness.

v  Aching all over.

v  Fever, wch may be slight to high (check your °C yourself).

v  Persistent loss of appetite.

v  Involuntary wt loss.

v  Increasing hair loss.

v  Recurring nose bleeds.

v  Sore é roof of mouth, wch burns é spicy foods.

v  Unexplained skin rash anywhere on the body.

v  Hives.

v  Sores on the skin

v  Painful joint(s)

v  Swollen joint(s)

v  Stiffness of joints é waking up in the morning

v  Chest pain wch incr. é breathing

v  Shortness of breath

v  Coughing up blood.

v  Persistent unusual headache.

v  Nausea or vomiting.

v  Recurring or persistent abd. pain.

v  Persistent, incr. swelling of feet & legs

v  Puffy eyelids.

v  Blood in the urine.

  

N.B. : “Silent Lupus:                 renoprotective meaning renoprotective antihypertensive drugs renoprotective ace inhibitors renoprotective antidiabetic drugs renoprotective agents renoprotective arbs renoprotective antihypertensive renoprotective action                    

                           {ptn. Clinically Normal + “Proliferative  les. é R. Biopsy.}.

Q.155. What are the serological parameters of clinical implications in S.L.E.?

*                  Anti-Sm. A.B.: (25% of cases) very specific to “Lupus nephritis” & greatly ass. é CNS, R. disease, more cutaneous mnf. &, cardiopulmonary mnf. & Worse prognosis. 

*                  Maternal (anti-Ro): Cardiac anomalies in newly born. 

*                  Anti-Ro Unique dermal form of Lupus é Psoriaform of Lupus.

*                  C3/C4 start decline before clinical flare, CH50 is better thn isolated C3 & C4.

*                  C3/CH50 may dcr. without clinical activity, é sev. dermatologic involvement.

Q.156. Which group of drugs can induce Lupus? 


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A. Drug-induced lupus: [Hydralazine- Procainamide-Penicillamine-Diltiazem-INH-Methyl dopa]use for > one m. or longer: can induce Lupus.

Q.157. How to manage?

A. History of drug intake and presence of (Anti-histone auto-A.B.), help in Dg.x. of drug-induced Lupus, which. can be reversed by stoppage the offending drug. The "gold standard" in Dg.X. is spontaneous resolution of the disease é 1-7 m. after the offending drug has been discontinued. Addition of NSAID & antimal-arials will help in clearance of constitutional & musculoskeletal  Sm.s rapidly. Steroids cn help in quick resolution of serositis.“Cytotoxic therapy” only needed in “Hydralazine -induced” vasculitis.

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

A.                 {Steroid therapy. + 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.  


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