All that wheezes is not

Ashtma!!!

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

• h/o asthma since 2011- under Dr Chitnis care.

• h/o nasal polypectomy at JHRC in May 2013Investigations – (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

On examination

• B P - 170/110• Maxillary sinus tenderness• Bilateral ronchi, crackles• No nasopharyngeal destructive lesions • No vasculitic rash

Investigations

• Hb -11.5 gms% • WBC -27670/c mm (Eosinophil count 57%)• Platelets - Adequate• ESR- 80 mm/hour • Blood urea - 91 mg%• Creatinine- 6.2mg%• Potassium - 5.5 mEq/L

• 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

To summarise-Young male with • new onset uncontrolled asthma• nasal polyposis• rapidly declining renal function • with hypertension• and persistent eosinophilia

Probable pulmonary renal syndromeANCA associated vasculitis

• 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

Final diagnosis

Churg Strauss Syndrome

• 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

• 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%

• 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)

• 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

Churg Strauss syndrome -1951 Dr. J Churg Dr. L Strauss

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

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

Natural history of diseaseProdormal 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

Vasculitic phase

a) General systemic disease • weight loss, rash ,myalgia• migratory nonerosive polyarthritis

b) Cardiac – • Pancarditis• Endomyocardial fibrosis, restrictive

cardiomyopathy• Coronary vasculitis- IHDLeading cause of mortality

b)Pulmonary involvement- • Diffuse alveolar hemorrhage or• Exudative eosinophil rich pleural effusion

c) Gastrointestinal – • Abdominal pain,• Ascites, • Diarrhea, and/or hematochezia

d) CNS-• Mononeuritis multiplex (2nd MC feature)• Cerebral vasculitis - hemorrhagic stroke

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 tractGranulomatous involvement of prostrate

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)

Treatment

Induction therapy • Corticosteroids and cyclophosphamide• Plasma exchange

Maintainance therapy• IV or oral Cyclophosphamide• Azathioprine • Mycophenolate mofetil• Biologics

Prognosis

• 5-year renal and patient survival is 65-75%• More the number of organs involved, worser

the outcome• ANCA negative vasculitishave more extrarenal organ manifestations, higher chance for relapse higher mortality

Spontaenous Retroperitoneal Hemorrhage

(SRH) in Dialysis patientsReview 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

• 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%.

• 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 microvasculaturec) Minimal trauma (cough, vomitting) can trigger

the processd) Uremic platelet dysfunction, peripheral

destruction and decreased production, HIT

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

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