NEPHROLOGY CASE PRESENTATION

• Presenting Complaint:– 56yr old female known to nephrology– Bought in by ambulance – found at home

cyanotic and gasping– At a recent clinic visit: diagnosed with a UTI

and started on oral antibiotics– Has since experienced SEVERE nausea and

vomiting limiting oral intake of food, fluids and medication

• Background Hx– Known with mixed connective tissue disease

with features of CREST syndrome– Followed up at nephrology for chronic renal

failure • Not on dialysis• Creatinine stable at 400umol/l• Complicated by anaemia and hypertension – both

appear well managed

– Known with severe oesophageal dysmotility– Treated at last clinic visit for an e coli UTI

(begun on cefuroxime three days ago)

• Current Medication

Sotalol 80mg po od

Enalapril 5mg po bd

Adalat XL 30mg po od

FeSO4 one tab daily

Omerprazole 20mg po od

Lasix 125mg po bd

Prednisone 10mg po od

Slow K II po bd

• On General Examination– Initial Assessment

• Acutely ill with respiratory distress

• Severe peripheral cyanosis – including ears and tip of nose

• Cold peripheries with impalpable peripheral pulses

• Drowsy/Encephalopathic

• BP 243/130

• Saturation un-recordable

• Conjunctival pallor

• no jaundice, oedema, lymphadenopathy

– Upon Re-Examination: Once stable the in ward• Sclerodactyly

• Telangiectasia

• Puckering of the skin around the mouth

• Cardio-respiratory Exam– Poorly palpable peripheral pulses

and severe cyanosis– Confirm BP of 243/130– Elevated JVP, unable to lie flat– No palpable P2, apex slightly laterally

displaced– Loud S3, no murmurs– Bilateral creps and exp wheeze extending from

the bases to the upper zones posteriorly– Still no Sats reading (blood gas drawn)

• Neurological Exam– GCS 14/15, marked drowsiness– Able to follow commands and appears to be

able to move all limbs against gravity– No facial asymmetry– Fundoscopy limited by severe distress

• Abdominal Exam– No organomegally– Non tender abdomen– No bruit

• Management in Referrals– Clinical decision to intubate pending the blood

gas– Managed as pulmonary oedema

• IV furosemide boluses and nitrates

• BP managed (single dose enalapril 10mg PO)

– Multi-consulted for admission for ventilation and BP control

• Investigations:– ECG: Sinus rhythm, No acute ST changes– Chest x ray: Confirms pulmonary oedema– Urine dipstix: Trace protein, + Nitrites, + Leuks– All others pending

• Differential Diagnosis?

• Hypertensive Emergency– Elevated BP with Acute Pulmonary oedema– Likely precipitated by inability to take

medication

• To exclude Scleroderma Renal Crisis

• Blood Results

Sodium 137 Potassium 5.6 Carbon dioxide 14Urea 21.2 Creatinine 444 Glucose 8.2 mmol/l

Calcium 2.38 mmol/l Albumin 33 g/l Phosphorus inorganic 2.41 mmol/l

Bilirubin 6 µmol/l Bilirubin conj 3 µmol/lTotal Protein 74 g/lAST 148 U/l ALT 108 U/lALP 95 U/l GGT 123 U/l LDH 313 U/l

CRP 23.7 mg/l White Cell Count 8.58 x 10^9/lHaemoglobin 12.3 g/dlPlatelets 178 x 10^9/l

• Progress in ICU– Responded well to BP control with IV labetolol– Kept on ACE inhibitor for management of

possible Scleroderma Renal Crisis– Pulmonary oedema resolved with PPV and BP

control– Creatinine remained stable, urine output

adequate– (Rising urea attributed to dehydration and

dipeptivan administration – resolved)

SCLERODERMA RENAL CRISIS

Introduction

• Renal involvement common in Systemic Sclerosis – usually manifesting as mild renal dysfunction

• Scleroderma Renal Crisis is a life threatening disease that develops in 10-20% of patients with Diffuse Cuntaneous form of Systemic Sclerosis

• (Much less frequent in Limited Cutaneous form)• High morbidity and mortality• Characterized by:

– Abrupt onset of moderate to severe hypertension– Mild proteinuria or few cells or casts– Progressive renal failure

General Renal Involvement

• One half of patients have renal involvement• Proteinuria, elevated creatinine, hypertension• Multifactorial

– Disease itself: lesion in interlobular arteries associated with chronic renal ischaemia

– Hypertension– Renal artery stenosis– PreRenal: Hypovolamia (GI invlovement, diuretic use,

heart failure)– Drugs: NSAIDS, cyclosporine, D-penacillamine

Scleroderma Renal Crisis

• Clinical Features– Early complication (usually within first 5 years)– Median occurrence 7.5 months after diagnosis– May be presenting manifestation– Acute renal failure usually in absence of

previous significant renal disease– Abrupt onset of moderate to marked

hypertension

• Clinical Features Contd– Urine sediment usually normal or mild

proteinuria with a few casts or cells– Microangiopathic haemolytic anaemia– Additional findings

• Heart failure

• Pulmonary oedema

• Features of malignant hypertension

Risk Factors

• Most NB risk factor - diffuse skin involvement

• Glucocorticoid use• Presence of autoantibodies to RNA

polymerase• ? Cyclosporin• New onset heart failure, pericardial

effusion, new onset anaemia

Pathology

• Primary changes located in the arcuate and interlobular arteries and the glomeruli

• Intimal proliferation and thickening

• Narrowing and obliteration of lumen

• Concentric/onion skin hypertrophy

• (similar to histopathological changes found in other organs)

Diagnosis

• Characteristic findings in a high risk patient– New onset BP >150/85mmHg– Progressive decline in renal function– Additional findings include

• Microangiopathic haemolytic anaemia• Retinal changes• Flash pulmonary oedema• Oliguria• Characteristic changes on renal biopsy

Treatment

• Mainstay: Prompt BP control (attempt to return BP to baseline within 72hrs)

• Optimal class = ACE Inhibitors• IV nitroprusside for malignant HPT with CNS

involvement• ARB’s would be expected to be effective but no

clinical trials have compared these to ACEI• IV prostacyclins (anecdotal observations of benefit)• Fish oil (theoretical benefit on haemodynamics and

antipalatelet properties)

Renal Outcomes

• Despite treatment with ACE 20-50% progress to end-stage renal disease

• If indicated either haemo- or peritoneal dialysis may be initiated

• Survival on dialysis of patients with systemic sclerosis is worse than those with other forms of end-stage renal disease

• Limited experience with renal transplant (often precluded due to severity of extra-renal manifestations)

• Survival is reduced compared to transplant recipients with other disorders

Prognosis

• Potentially life-threatening complication

• Prior to ACEI use most died within 1 year

• 76% one year survival in those treated with ACEI (compared to 28% with other anti-hypertensives)