2. Mr R.T, 23 Yrs male, referred for evaluation of high
creatinine with new onset hypertension on June
10, 2013
On enquiry he had -
• Breathlessness and dry cough
• Pain in maxillary sinus area
• Generalized myalgia
• Loose motions (multiple episodes)
• Weight loss of 9 kg in 6 months
• No fever, no oligoanuria
3. • h/o asthma since 2011- under Dr Chitnis care.
• h/o nasal polypectomy at JHRC in May 2013
Investigations – (May 2013)
Urine R/M -
1+ Albumin with 20 – 25 RBC/HPF
CBC- Hb 14.8 gms%
WBC 11,740( Eosinophil count 25.5%)
Renal function test was not done nor was he
investigated during that admission
4. On examination
• B P - 170/110
• Maxillary sinus tenderness
• Bilateral ronchi, crackles
• No nasopharyngeal destructive lesions
• No vasculitic rash
6. • Albumin 3.2 gms%,
• Calcium 8.6 mg%, Uric Acid 9.2 mg%.
• Urine (r) ++++ Albumin. 60-70 RBC/HPF
• Urine Protein/Creatinine ratio 8.2
• ANA – 1:80 dilution – weak positive
• p/c ANCA – negative
• Anti GBM- negative
• C3 and C4 normal
• Vitamin D < 8 ng/ml
• USG normal size kidneys
7. To summarise-
Young male with
• new onset uncontrolled asthma
• nasal polyposis
• rapidly declining renal function
• with hypertension
• and persistent eosinophilia
Probable pulmonary renal syndrome
ANCA associated vasculitis
8. • Admitted in hospital
• B P controlled with oral antihypertensives
• Biopsy done under ultrasound guidance.
• Uneventful, check ultrasound normal
• Creatinine increased to 8 mg% next day
• Started on pulse Steroid Therapy – 1 Gm IV for
three days after biopsy
13. • Patient was started on Plasma exchange on
alternate days and planned to do this for five
times
• After 4th Plasma Exchange, almost 10 days
after kidney biopsy, potassium was found to
be 6.5 mEq/L refractory to medical mgt.
• Hence hemodialysis done following Plasma
Exchange which he tolerated well
14. • Late in evening patient complained of back pain
and abdominal pain
• Not responding to PPI or Tramadol
• USG of abdomen done was normal and so were
Amylase and Lipase
• Next day Hb dropped by 1.5 gms%
15. • Hence CT scan ordered which showed large
retroperitoneal bleed
• PT/PTT/INR deranged
• Patient transferred to ICU for further
management
• Patient treated with 6 units blood transfusions
along with FFP
• Hb remained low in spite of blood transfusion
• Patient’s family contacted
• Decided to give rHu Factor VII (Novoseven)
16. • Two doses of recombitant factor VII given
• Bleeding stopped and Hb remained stable
• Serum Creatinine was 8 mg% prior to stating
IV pulse steroid, now settled around 4.5 to 5
mg%
• No further plasma exchanges were given
• Later switched over to Defcort 36 mg daily
• Patient discharged after a month’s
hospitalisation
18. Described 13 patients on autopsy who had -
a) new/ late onset asthma and/or allergic rhinitis,
b) hypereosinophilia
c) culminating in frank necrotising vasculitis of
small and medium-sized vessels.
Also called as
Allergic angiomatosis and granulomatosis
19. Epidemiology
• Global occurrence
• Rare disease.
• Estimated annual incidence 1-2 per milllion
• All ages affected , max in 3rd and 4th decade
• M:F =1.2 : 1
20. Natural history of disease
Prodormal phase-
a) Allergic rhinitis -
First manifestation , MC seen in 70% of cases.
Nasal polyposis, recurrent sinusitis
b) Asthma –
Late onset, severe, no family history or allergen identified
c) Eosinophilia -
• Loffler’s pneumonia , Chronic Eosinophilic Pneumonia.
• GIT- may present as eosinophilic gastroenteritis
21. Vasculitic phase
a) General systemic disease
• weight loss, rash ,myalgia
• migratory nonerosive polyarthritis
b) Cardiac –
• Pancarditis
• Endomyocardial fibrosis, restrictive cardiomyopathy
• Coronary vasculitis- IHD
Leading cause of mortality
23. e) Renal manifestation –
• Microscopic hematuria
• Nephritic range proteinuria (at times nephrotic)
• Mild azotemia to rapidly deteriorating renal
function requiring permanent RRT
• Obstuctive uropathy due to
Vasculitic involvement of ureters and lower
urogenital tract
Granulomatous involvement of prostrate
24. Investigation
• Peripheral and tissue eosinophilia, Increased IgE
• P- ANCA / Myeloperoxidase positive- 70 %
• C- ANCA/ Anti PR3 – 10 %
• ANCA negative – 20 %
Kidney biopsy
• Focal segmental GN or Crescentric GN
• Diffuse necrotizing GN
• No immune complex deposits
(hence called pauci-immune GN)
25. Treatment
Induction therapy
• Corticosteroids and cyclophosphamide
• Plasma exchange
Maintainance therapy
• IV or oral Cyclophosphamide
• Azathioprine
• Mycophenolate mofetil
• Biologics
26. Prognosis
• 5-year renal and patient survival is 65-75%
• More the number of organs involved, worser
the outcome
• ANCA negative vasculitis
have more extrarenal organ manifestations,
higher chance for relapse
higher mortality
27. Spontaenous
Retroperitoneal Hemorrhage
(SRH) in Dialysis patients
Review of MEDLINE database ( 1971 – 2010)
- Malek et al Clin Neph 2010
• 34 publications, 55 reported cases only
• 95% on hemodialysis , 5% on peritoneal dialysis
• MC source of bleed - Kidney (87.8%)
• MC cause - ADPKD
• 91.8 % were on some anticoagulation
28. • Risk of bleed-
With Heparin (UFH > LMWH) 5 times greater
than Warfarin
Rarely clopidogrel
• Treatment modality used –
a) Surgery – 49%
b)Conservative- 33.3%
c)Angioembolization – 17.7
• Mortality rate was 18.3%.
29. • Corellation with dose of heparin – No
• Duration of dialysis – More in chronic RRT
(Median duration – 7 yrs)
• Why SRH occurs in dialysis patient?
a) Persistent anticoagulant exposure
b) Diffuse atherosclerosis of microvasculature
c) Minimal trauma (cough, vomitting) can trigger
the process
d) Uremic platelet dysfunction, peripheral
destruction and decreased production, HIT
30. Management-
a) Conservative approach best if patient is
hemodynamically stable.
Rationale being the “tamponade effect”
May give -
• FFP
• PCC (prothrombin complex concentrate)
• Recombitant factor VII
Angioembolisation/Surgery only if deteriorating
31. b) Angioembolisation –
• Best modality if SRH is iatrogenic in kidney
patients i.e following translumbar, femoral
catheter insertion or post kidney biopsy
• In SRH , doubtful diagnostic or therapeutic utility
as it is an angiographically occult diffuse
microvascular condition
• To be do if there is active extravasation of contrast
on CT
c) Surgical exploration/ CT guided drainage-
• Worsening GC
• Abdominal compartment syndrome
