PMR Classification Criteria

LARGE VESSEL VASCULITIS
Kuntal Chakravarty
S El naas & F Alyas
David Jayne

1: A patient with postural paralysis
2: A patient with acute SOB
3: A patient with pain in upper limbs

POSTURAL PARALYSIS

Mrs C P 60 yrs. Insurance Admin.
GP: Sept’ 11
Anorexia and Weight Loss – 9kg in 6/12
Anemia - 8.7 gm / dl
Generalized fatigue and tiredness
ESR – 106 mm 1 st hour
OGD & COLONOSCOPY ( 2 week)

Past Medical, Family & Social History
2006 - Chronic headache
( MRA,MRI-NAD)
F/H : Sister ( 50 YRS) fatal SAH
Smoker - < 10 cigs a day
Not on regular medication

Medical Admission – 21.9.11
Several collapses since 16.9.11
No prodrome, aura or incontinence
Mild hyperventilation
Proximal weakness in limbs
Weakness improved in seconds on lying flat
Post recovery: No loss of memory or
power in limbs
No h/o claudication in limbs

Clin. Exam/ Investigations
No postural drop in BP
No difference in pulse / BP in limbs
No loss of consciousness / neurological deficit
ECG/TOE /CT CAP – NAD
MRI , NCS – NAD
OGD/COLONOSCOPY –NAD
U&E,Cortisol,TFT, TPHA & Viral serology,HIV
CK, Igs/SPEP, ANCA, Compl ,RF, acL – NEG
Haematinics : NAD blood cultures – neg
HB: 8.9 ESR : 120 CRP : >100

RHEUMATOLOGY - OCT ’11
PET Scan

20.09.2011

CT Aortogram

Treatment – Oct 2011
Initial IV MP Pulses x 3
Oral prednisolone : 60 mg tapering dose
Significant Clinical improvement matched
with drop in ESR / CRP

IVMP
Steroid taken off
(Compliance issue )
Steroid taken off
(Compliance issue )
IVMP
Steroid taken off
(Compliance issue )
IVMP

Progress (DJ/KC)- Feb ‘12
Patient declined to continue on steroid
Relapse of fatigue, tearfulness, mood
swings and occasional collapse
BP – 160/90 ; No postural drop/ No pulse
difference between limbs
ESR – rising (80)
U/S of temporal /axillary / carotid
thickening
TA Biopsy - Neg.

Longitudinal US of carotid

Mrs CP 60 yrs
Presented with systemic symptoms ,
anaemia, raised ESR, CRP, recurrent
collapse on standing with no neurological
abnormality
PET / Aortogram Large vessel vasculitis
Patient compliance ?? With steroid
?? TOCILIZUMAB

Acute SOB

K R 27 yrs old, mother of three
FEBRUARY- 2012
• Acute onset of SOB - few days
• Chest tightness & Palpitations – 4 / 52
• Pain and paraesthesia in (R) arm
• Abdominal pain & Neck pain

Past Medical History
Hypertension > 9 months
complicated last pregnancy 7/12 , pre eclampsia
2 previous uneventful pregnancies
Chronic Recurrent Multifocal Osteomyelitis
under care of rheum/ortho
previous bisphosphonate treatment
Non smoker & NO F/H of any CV Disease

Initial Assessment by GP
Pulse : 98/mt regular ; HS : NAD
BP– Lt arm : 200 / 120 mm Hg
Rt arm : 160 / 100 mm Hg
GP : ? Coarctation of Aorta/ Cardiac failure
Immediate request for CT Scan of chest

Examination in A/E & MAU
( Documentation in case notes )
• Vital Signs
temp 36 C
Pulse 80/min, regular
BP 140/80mmHg (Left arm only )
RR 16/min and Sats: 96% in room air
• CVS examination
Rt Arm : absent Radial & Brachial pulses
BP L arm – 200/100 mmHg
R arm – 140/80 mmHg
Prominent Renal Bruits noted
weak femoral pulses , palpable Dorsalis Pedis
normal Heart sounds & NO sign of heart failure

Medications
• Labetalol 100mg BD
• Bendroflumethiazide 2.5mg OD
• Co-codamol & Gaviscon PRN

Initial Investigations: Hospital
• Hb: 10.7gms; WCC 9.9x10/l , Platelets: 497
• ESR : 110 mm 1 st hr (20); CRP : 124 (60,
• LFT: ALP 195( 128);Protein 92 (83), glob:49(36)
Trop I

Imaging Investigation
CT Chest showing - diffuse
concentric thickening of
proximal part of arch of
aorta seen

Further imaging
CT Chest/Abdo showing - diffuse concentric thickening of proximal part
of arch of aorta seen. Diffuse thickening also extend to involve the
descending thoracic aorta and visualised upper part of abdominal aorta

Rheumatology : March 2012
• H/O ABSENT PULSE in Right radial since
childhood & Carotidynia
• MRA & CT Angio
• Diagnosis: Large vessel vasculitis
• Renal Artery Stenosis – reno-vascular HTN

MRA – Occlusion of right brachiocephalic, subclavian and common carotid arteries.
Significant narrowing of celiac and bilateral renal arteries with near total occlusion of
superior mesentric arteries

CT Aortogram

Management
• Oral prednisolone , Aspirin & Lansoprazole
• Renal Team : amlodipine
• Review in Rheumatology Clinic
• Review 6 weeks with vascular surgeons
• Rescan

KR-The Inflammatory Markers

FOLLOW UP – Mid April ,2012
• WELL with lots of questions (Dr Google)
• UPSET : Cushingoid on 60mg / day – 1 month
• BP: 130/90 (R) & 146/84(L)
• Hb:11.9 gms/dl; WCC: 16.3x10 /l; (neut 11.9)
• Platelets: 417, ESR: 49; CRP:

Pain in the upper limb

Mrs C A. 61yrs house wife
GP : 24 th MARCH 2009
Vascular Surgeon:
‘ This patient seems to have a problem
with blood supply to her left arm – it feels
cold and BP was un recordable’
PMH: Hypertension, Hypothyroid
Hypercholesterolaemia, Bilateral THR
Drugs: Amlodipine,T4, Aspirin, simvastatin
FBC : Normal
CXR – Normal , ECG - Normal

Vascular Surgeon: 27.4.09
Claudication symptoms in R>L upper limb
No peripheral pulses in both upper limbs
Carotid - poor pulsation
DUPLEX : Occlusion of left Axillary artery
Organize CTA & MRA

Admission for endarterectomy-
27 th JULY 2009
Absence of pulses in both radial
BP difference between two limbs
Blood test:
Hb: 10.9 gms/dl , ESR: 87; CRP: 45 (

MRI-
Axial T2

CT aortogram

MRA - 6 th August 2009
Int. Carotid Stenosis: 90% stenosed(R)
80% stenosed(L)
Common Carotid & Subclavian (L):Stenosed
Axillary (L): Blocked
C/0 : (L) Temporal Headache
RHEUMATOLOGY: Jaw claudication
Temporal artery U/S & Biopsy

PROGRESS: 2010-2012
Remains well on 5mg pred alternate day
NO limiting symptoms
Vascular Surgeons:Repeat Vascular study
NO SURGICAL INTERVENTION

Local data
Population : 780 000
Diverse and deprived
23 patients in the last 17 yrs
High incidence of TB @ DH
BUT
White Caucasians
No TB association
Unusual presentation

Intractable back pain

Mr A.H – 50 years Carpenter
• Feb’ 2008
• Back pain - 4-6 weeks
• No bladder / bowel symptoms
• Occasional paraesthesia on the right leg
• No history of any recent injury

• G P Orthopaedic Surgeon
• C T Scan of lumbo-sacral spine
• MRI Scans of the lumbo-sacral spine x 2
• Epidurals ?? ‘ Can’t remember doctor’

• NO IMPROVEMENT AFTER 1- year
• Persistent Back & abdo pain
• Anorexia , weight loss- 12 kgs in 1 – yr
• Cancerophobia
• Referred to Gen Surgeon

Investigations
• Hb: 12 gms/dl, WCC – 13 x10 9 /l, ESR-94
• U&E, LFT, AMYLASE – NAD
• Prostatic specific antigen - Normal
• C 199
- Normal

Further intervention
• Open laparotomy & biopsy
• Non malignant tissue
• Refer to Uro surgeons Rheumatologist

RHEUMATOLOGY - PP
• Looked ill , depressed , ? Cancer
• Mild loss of lumbar lordosis
• Raised JVP & pedal pitting

Further investigations
• ECHO – NAD
• Immunoglobulin assay: IgA – 5.46 (< 4.2)
• HLA B27 +
• Auto antibodies:ANF, DNA,ENA, ANCA-Negati

50 yrs old man with weight loss , anorexia
?? A S
P E T Scan

Pre-treatment

Treatment
• Oral Prednisolone : Tapering Dose – 1yr
• Dramatic improvement

Post-treatment

Progress
• Patient discontinued steroids after 9/ 12
• 2012 - NOT ON ANY DRUG.
• Remains Well