PMR Classification Criteria
PMR Classification Criteria
PMR Classification Criteria
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LARGE VESSEL VASCULITIS<br />
Kuntal Chakravarty<br />
S El naas & F Alyas<br />
David Jayne
1: A patient with postural paralysis<br />
2: A patient with acute SOB<br />
3: A patient with pain in upper limbs
POSTURAL PARALYSIS
Mrs C P 60 yrs. Insurance Admin.<br />
GP: Sept’ 11<br />
Anorexia and Weight Loss – 9kg in 6/12<br />
Anemia - 8.7 gm / dl<br />
Generalized fatigue and tiredness<br />
ESR – 106 mm 1 st hour<br />
OGD & COLONOSCOPY ( 2 week)
Past Medical, Family & Social History<br />
2006 - Chronic headache<br />
<br />
( MRA,MRI-NAD)<br />
F/H : Sister ( 50 YRS) fatal SAH<br />
Smoker - < 10 cigs a day<br />
Not on regular medication
Medical Admission – 21.9.11<br />
Several collapses since 16.9.11<br />
No prodrome, aura or incontinence<br />
Mild hyperventilation<br />
Proximal weakness in limbs<br />
Weakness improved in seconds on lying flat<br />
Post recovery: No loss of memory or<br />
power in limbs<br />
No h/o claudication in limbs
Clin. Exam/ Investigations<br />
No postural drop in BP<br />
No difference in pulse / BP in limbs<br />
No loss of consciousness / neurological deficit<br />
ECG/TOE /CT CAP – NAD<br />
MRI , NCS – NAD<br />
OGD/COLONOSCOPY –NAD<br />
U&E,Cortisol,TFT, TPHA & Viral serology,HIV<br />
CK, Igs/SPEP, ANCA, Compl ,RF, acL – NEG<br />
Haematinics : NAD blood cultures – neg<br />
HB: 8.9 ESR : 120 CRP : >100
RHEUMATOLOGY - OCT ’11<br />
PET Scan
20.09.2011
CT Aortogram
Treatment – Oct 2011<br />
Initial IV MP Pulses x 3<br />
Oral prednisolone : 60 mg tapering dose<br />
Significant Clinical improvement matched<br />
with drop in ESR / CRP
IVMP<br />
Steroid taken off<br />
(Compliance issue )<br />
Steroid taken off<br />
(Compliance issue )<br />
IVMP<br />
Steroid taken off<br />
(Compliance issue )<br />
IVMP
Progress (DJ/KC)- Feb ‘12<br />
Patient declined to continue on steroid<br />
Relapse of fatigue, tearfulness, mood<br />
swings and occasional collapse<br />
BP – 160/90 ; No postural drop/ No pulse<br />
difference between limbs<br />
ESR – rising (80)<br />
U/S of temporal /axillary / carotid <br />
thickening<br />
TA Biopsy - Neg.
Longitudinal US of carotid
Mrs CP 60 yrs<br />
Presented with systemic symptoms ,<br />
anaemia, raised ESR, CRP, recurrent<br />
collapse on standing with no neurological<br />
abnormality<br />
PET / Aortogram Large vessel vasculitis<br />
Patient compliance ?? With steroid<br />
?? TOCILIZUMAB
Acute SOB
K R 27 yrs old, mother of three<br />
FEBRUARY- 2012<br />
• Acute onset of SOB - few days<br />
• Chest tightness & Palpitations – 4 / 52<br />
• Pain and paraesthesia in (R) arm<br />
• Abdominal pain & Neck pain
Past Medical History<br />
Hypertension > 9 months<br />
complicated last pregnancy 7/12 , pre eclampsia<br />
2 previous uneventful pregnancies<br />
Chronic Recurrent Multifocal Osteomyelitis<br />
under care of rheum/ortho<br />
previous bisphosphonate treatment<br />
Non smoker & NO F/H of any CV Disease
Initial Assessment by GP<br />
Pulse : 98/mt regular ; HS : NAD<br />
BP– Lt arm : 200 / 120 mm Hg<br />
Rt arm : 160 / 100 mm Hg<br />
GP : ? Coarctation of Aorta/ Cardiac failure<br />
Immediate request for CT Scan of chest
Examination in A/E & MAU<br />
( Documentation in case notes )<br />
• Vital Signs<br />
temp 36 C<br />
Pulse 80/min, regular<br />
BP 140/80mmHg (Left arm only )<br />
RR 16/min and Sats: 96% in room air<br />
• CVS examination<br />
Rt Arm : absent Radial & Brachial pulses<br />
BP L arm – 200/100 mmHg<br />
R arm – 140/80 mmHg<br />
Prominent Renal Bruits noted<br />
weak femoral pulses , palpable Dorsalis Pedis<br />
normal Heart sounds & NO sign of heart failure
Medications<br />
• Labetalol 100mg BD<br />
• Bendroflumethiazide 2.5mg OD<br />
• Co-codamol & Gaviscon PRN
Initial Investigations: Hospital<br />
• Hb: 10.7gms; WCC 9.9x10/l , Platelets: 497<br />
• ESR : 110 mm 1 st hr (20); CRP : 124 (60,<br />
• LFT: ALP 195( 128);Protein 92 (83), glob:49(36)<br />
Trop I
Imaging Investigation<br />
CT Chest showing - diffuse<br />
concentric thickening of<br />
proximal part of arch of<br />
aorta seen
Further imaging<br />
CT Chest/Abdo showing - diffuse concentric thickening of proximal part<br />
of arch of aorta seen. Diffuse thickening also extend to involve the<br />
descending thoracic aorta and visualised upper part of abdominal aorta
Rheumatology : March 2012<br />
• H/O ABSENT PULSE in Right radial since<br />
childhood & Carotidynia<br />
• MRA & CT Angio<br />
• Diagnosis: Large vessel vasculitis<br />
• Renal Artery Stenosis – reno-vascular HTN
MRA – Occlusion of right brachiocephalic, subclavian and common carotid arteries.<br />
Significant narrowing of celiac and bilateral renal arteries with near total occlusion of<br />
superior mesentric arteries
CT Aortogram
Management<br />
• Oral prednisolone , Aspirin & Lansoprazole<br />
• Renal Team : amlodipine<br />
• Review in Rheumatology Clinic<br />
• Review 6 weeks with vascular surgeons<br />
• Rescan
KR-The Inflammatory Markers
FOLLOW UP – Mid April ,2012<br />
• WELL with lots of questions (Dr Google)<br />
• UPSET : Cushingoid on 60mg / day – 1 month<br />
• BP: 130/90 (R) & 146/84(L)<br />
• Hb:11.9 gms/dl; WCC: 16.3x10 /l; (neut 11.9)<br />
• Platelets: 417, ESR: 49; CRP:
Pain in the upper limb
Mrs C A. 61yrs house wife<br />
GP : 24 th MARCH 2009<br />
Vascular Surgeon:<br />
‘ This patient seems to have a problem<br />
with blood supply to her left arm – it feels<br />
cold and BP was un recordable’<br />
PMH: Hypertension, Hypothyroid<br />
Hypercholesterolaemia, Bilateral THR<br />
Drugs: Amlodipine,T4, Aspirin, simvastatin<br />
FBC : Normal<br />
CXR – Normal , ECG - Normal
Vascular Surgeon: 27.4.09<br />
Claudication symptoms in R>L upper limb<br />
No peripheral pulses in both upper limbs<br />
Carotid - poor pulsation<br />
DUPLEX : Occlusion of left Axillary artery<br />
Organize CTA & MRA
Admission for endarterectomy-<br />
27 th JULY 2009<br />
Absence of pulses in both radial<br />
BP difference between two limbs<br />
Blood test:<br />
Hb: 10.9 gms/dl , ESR: 87; CRP: 45 (
MRI-<br />
Axial T2
CT aortogram
MRA - 6 th August 2009<br />
Int. Carotid Stenosis: 90% stenosed(R)<br />
<br />
80% stenosed(L)<br />
Common Carotid & Subclavian (L):Stenosed<br />
Axillary (L): Blocked<br />
C/0 : (L) Temporal Headache<br />
RHEUMATOLOGY: Jaw claudication<br />
Temporal artery U/S & Biopsy
PROGRESS: 2010-2012<br />
Remains well on 5mg pred alternate day<br />
NO limiting symptoms<br />
Vascular Surgeons:Repeat Vascular study<br />
NO SURGICAL INTERVENTION
Local data<br />
Population : 780 000<br />
Diverse and deprived<br />
23 patients in the last 17 yrs<br />
High incidence of TB @ DH<br />
BUT<br />
White Caucasians<br />
No TB association<br />
Unusual presentation
Intractable back pain
Mr A.H – 50 years Carpenter<br />
• Feb’ 2008<br />
• Back pain - 4-6 weeks<br />
• No bladder / bowel symptoms<br />
• Occasional paraesthesia on the right leg<br />
• No history of any recent injury
• G P Orthopaedic Surgeon<br />
• C T Scan of lumbo-sacral spine<br />
• MRI Scans of the lumbo-sacral spine x 2<br />
• Epidurals ?? ‘ Can’t remember doctor’
• NO IMPROVEMENT AFTER 1- year<br />
• Persistent Back & abdo pain<br />
• Anorexia , weight loss- 12 kgs in 1 – yr<br />
• Cancerophobia<br />
• Referred to Gen Surgeon
Investigations<br />
• Hb: 12 gms/dl, WCC – 13 x10 9 /l, ESR-94<br />
• U&E, LFT, AMYLASE – NAD<br />
• Prostatic specific antigen - Normal<br />
• C 199<br />
- Normal
Further intervention<br />
• Open laparotomy & biopsy<br />
• Non malignant tissue<br />
• Refer to Uro surgeons Rheumatologist
RHEUMATOLOGY - PP<br />
• Looked ill , depressed , ? Cancer<br />
• Mild loss of lumbar lordosis<br />
• Raised JVP & pedal pitting
Further investigations<br />
• ECHO – NAD<br />
• Immunoglobulin assay: IgA – 5.46 (< 4.2)<br />
• HLA B27 +<br />
• Auto antibodies:ANF, DNA,ENA, ANCA-Negati
50 yrs old man with weight loss , anorexia<br />
?? A S<br />
P E T Scan
Pre-treatment
Treatment<br />
• Oral Prednisolone : Tapering Dose – 1yr<br />
• Dramatic improvement
Post-treatment
Progress<br />
• Patient discontinued steroids after 9/ 12<br />
• 2012 - NOT ON ANY DRUG.<br />
• Remains Well