Dr Kusala S. Gunasekara MBBS(Col),MD(Med),MRCP(UK) Acting Consultant Rheumatologist DGH-Matale

Transcription

1 Dr Kusala S. Gunasekara MBBS(Col),MD(Med),MRCP(UK) Acting Consultant Rheumatologist DGH-Matale

2 Patient 67 yr old female First presentation to the rheumatology unit in May 2013 Referred by GP as the patient complained of having bilateral shoulder joint, hip joint and proximal leg pain. Associated with mild stiffness of the bilateral shoulder joints in the morning.

3 No small joint involvement. No joint swelling. No constitutional symptoms Treated with Naproxen by the GP but no significant relief gained. Past Medical History Bronchial asthma, Hypothyroidism,Hyperlipidaemia.

4 Examination Shoulder joint Left side limited abduction and internal rotation. Right side full range of movement, no tenderness. Hip joint unremarkable No active synovitis No proximal muscle weakness

5 Investigations ESR 42 CRP 20 Others including rheumatoid screening autoantibody profile negative or normal. X ray hip joints NAD Provisional diagnosis left side rotator cuff tendinitis. Treatment left side steroid injection and physiotherapy.

6 Second clinic visit June 2013 Complained of worsening of pain in the shoulder and hip joints. Significant morning stiffness in shoulder and hip joints. Disturbances to daily activities like dressing and grooming. No other joint involvement. Constitutional symptoms LOA, malaise, generalized weakness. Denied GCA symptoms such as headache, scalp tenderness, jaw claudication, visual disturbances.

7 Examination B/L shoulder joints Abduction 90, Flexion 100. Hips good ROM Other joints no active synovitis. Normal proximal muscle power. Diagnosis Polymyalgia Rheumatica. Treatment started on prednisolone 20mg daily and planned to taper it off according to response.

8 August 2013 On Prednisolone 5mg daily. Complete resolution of symptoms. Good ROM in bilateral shoulder joints ESR 18 Planned to reduce the prednisolone further and completely stop it.

9 February 2014 Had a flare of symptoms in December Had increased prednisolone 10 mg daily with good response ESR 35 CRP -25 Planned to reduce prednisolone by 1mg every month.

10 June 2014 Patient had been referred to vascular surgeon by GP as she complained of claudications in lower limbs esp. in the calf. Each claudication was described to be induced by walking m distance. Also had jaw claudication. On ex unable to feel popliteal and pedal pulses bilaterally. Equal radial and brachial pulses.

11 Diagnosis -? Peripheral vascular disease Investigations CT angiogram bilateral diffuse SFA narrowing with tight focal stenosis. Aorta and iliac arteries normal. Planned femoral artery angioplasty.

12 August 2014 Patient reports difficulty in reducing prednisolone less than 7mg daily due to flare up in polymyalgia rheumatica symptoms. No GCA features. ESR 34 CRP 54 Treatment planned to start on Methotrexate or Azathioprine.

13 October 2014 On prednisolone 10 mg daily. Continue to have pain and stiffness in shoulder and hip girdles, lower limb claudication, night sweats. Ex upper limb pulses were felt. Right arm BP 152/66 Left arm BP 142/75 Ix CRP 65, ESR 46

14 ESR

15 CRP

16 Patient is having. Persistent PMR symptoms resistant to steroids. Prominent constitutional symptoms. Raised inflammatory markers. Clinical and radiological features suggestive of large vessel involvement.

17 Diagnosis -? Large vessel vasculitis CT PET scan Widespread abnormal increased linear uptake in walls of large arteries suggestive of large vessel vasculitis.

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19 November 2014 Increased prednisolone dose up to 60mg daily. Left superficial femoral artery angioplasty was performed with significant improvement in claudication and increased walking distance. Plan taper off prednisolone. added Azathioprine. Was referred to specialized vasculitis unit for further management and follow up.

20 Differential diagnosis When large-artery inflammation is suspected with symptoms, investigations and imaging. Both primary and secondary large-vessel vasculitis have to be considered. Primary large-vessel vasculitis mainly consists of GCA and Takayasu arteritis. Secondary large-vessel vasculitis is less common and may be caused by infections such as HIV, syphilis, tuberculosis, or hepatitis or may occur in systemic inflammatory diseases such as rheumatoid arthritis, Behçet s disease, relapsing polychondritis, systemic lupus erythematosus, sarcoidosis.

21 Polymyalgia rheumatica Inflammatory rheumatic condition characterized clinically by aching and morning stiffness in the shoulders, hip girdle, and neck. It can be associated with giant cell (temporal) arteritis and the two disorders may represent different manifestations of a shared disease process. PMR occurs in about 50 percent of patients with GCA, while approximately 15 to 30 percent of patients with PMR eventually develop GCA.? Sub clinical vasculitis.

22 PR-Diagnosis criteria Age 50 years or older at disease onset. Bilateral aching and morning stiffness (lasting 30 minutes or more) persisting for at least one month. The stiffness should involve at least two of the following three areas: neck or torso, shoulders or proximal regions of the arms, and hips or proximal aspects of the thighs. Erythrocyte sedimentation rate 40 mm/h.

23 BSR and BHPR guidelines for the management of Polymyalgia rheumatica Bhaskar Dasgupta1, Frances A. Borg1, Nada Hassan1, Kevin Barraclough2, Brian Bourke3, Joan Fulcher4, Jane Hollywood1, Andrew Hutchings5. Rheumatology Advance Access published November 12, 2009 The suggested regimen is: Daily prednisolone 15mg for 3 weeks Then 12.5mg for 3 weeks Then 10mg for 4 6 weeks Then reduction by 1mg every 4 8 weeks

24 Giant cell arteritis Chronic vasculitis of large and medium vessels leading to granulomatous inflammation histologically. Predominantly affects the cranial branches of arteries arising from the arch of the aorta. Incidence is reported as 2.2/ patient. Age >50 years. Incidence rates appear higher in northern climates. Both GCA and PMR often occur together. There are suggestions that the underlying pathophysiology is the same. Localized headache, jaw or arm claudication, visual symptoms, and any unusual pain in the face, throat, or tongue, any of which would suggest the possibility of GCA.

25 The American College of Rheumatology (ACR) classification criteria for GCA. Age at disease onset 55 years. New headache: new onset of or new type of localized pain in the head. Temporal artery abnormality: temporal artery tenderness to palpation or decreased pulsation. Elevated ESR: ESR55 mm/h. Abnormal artery biopsy: biopsy specimen with artery showing vasculitis characterized by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells. A patient shall be said to have GCA if at least three of these five criteria are present. The presence of any three or more criteria yields a sensitivity of 93.5% and a specificity of 91.2%.

26 GCA-Ix Temporal artery biopsy-gold standard Early TAB in all patients with suspected cranial GCA. This should be performed preferably within 1 week of starting glucocorticosteroids. However, reports suggest that TAB may remain positive for 2 to 6 weeks following initiation of glucocorticosteroids and glucocorticosteroids should not be delayed while awaiting TAB. TAB may be negative in some patients with GCA, due to the presence of skip lesions and suboptimal biopsy and prior steroid treatment.

27 BSR and BHPR Guidelines for the management of giant cell arteritis Bhaskar Dasgupta1, Frances A. Borg1, Nada Hassan1, Leslie Alexander1, Kevin Barraclough2, Brian Bourke3, Joan Fulcher4, Jane Hollywood1, Andrew Hutchings5, Pat James4, Valerie Kyle6, Jennifer Nott7, Michael Power8 and Ash Samanta9 Prednisolone 40 to 60mg (not <0.75 mg/kg) daily until resolution of symptoms and laboratory abnormalities. Complicated GCA: Evolving visual loss or history of amaurosis fugax: i.v. methylprednisolone 500mg to 1 g daily for 3 days then 40 to 60mg prednisolone (not <0.75 mg/kg) continued for 4 weeks (until resolution of symptoms and laboratory abnormalities). Then dose is reduced by 10mg every 2 weeks to20 mg daily. Then by 2.5mg every 2-4 weeks to 10 mg daily. Then by 1mg every 1 to 2 months provided there is no relapse. The early introduction of MTX or alternative immunosuppressant should be considered as adjuvant therapy.

28 Takayasu Arteritis Chronic vasculitis of unknown etiology. Primarily affects the aorta and its primary branches. ACR diagnostic criteria. Age at disease onset 40 years. Claudication of the extremities. Decreased pulsation of one or both brachial arteries. Difference of at least 10 mmhg in systolic blood pressure between the arms. Bruit over one or both subclavian arteries or the abdominal aorta. Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities. Patients are said to have Takayasu arteritis if at least three of the six criteria are present.

29 TA-Ix Angiograpy-Gold standard Because of the central location of the arteries involved by Takayasu arteritis, arterial biopsy is generally not an option. Thus, imaging of the aorta and major arteries is usually relied upon to confirm the diagnosis of Takayasu arteritis. The arteriographic changes with contrast angiography tend to be most pronounced in the region of the aortic arch and its primary branches, although involvement may be most prominent or limited to the more distal aorta and its branches. The primarily abnormalities are smooth-walled, tapered, focal, or narrowed areas with some areas of dilation. Can t differentiate active vs inactive disease.

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31 GCA vs TA Finding Giant cell arteritis Takayasu arteritis Female-to-male ratio 3 : 2 7 : 1 Age at onset >50 years <40 years Primary vessels involved Histopathology External carotid artery branches Granulomatous inflammation Aorta and branches Granulomatous inflammation Response to corticosteroids Excellent Excellent Course Self-limited Chronic

32 Diagnosis? Takayasu arteritis? Large vessel involvement in GCA (Extracranial GCA) Age 67 years Prominent Polymyalgia rheumatica symptoms

33 Extracranial GCA Large-artery involvement in patients with or without clinically apparent temporal arteritis (cranial GCA). Patients with solitary extracranial GCA often present with nonspecific signs and symptoms, high inflammatory markers and vascular manifestations mostly secondary to stenosis. It can also occur during corticosteroid decrease or withdrawal in patients with a known GCA. Complications consist of aortic aneurysm or dissection (mainly the ascending aorta), aortic arch syndrome, arm claudication and posterior stroke.

34 Extracranial GCA-Clinical features Non-specific symptoms are considered to be more common including malaise, fever (10-61%), weight loss (20%), anorexia, polymyalgia (0-40%) and muscle weakness (6.3%). Vascular symptoms such as limb claudication, Raynaud s phenomenon, digital ischaemia, decreased pulses, arterial bruits and signs of cerebral ischaemia are more specific, but are considered less common. Extracranial GCA should be considered in elderly patients presenting with elevated inflammatory markers in combination with either non-specific symptoms or peripheral arterial disease. Refractory Cranial GCA disease should also raise suspicion of extracranial involvement.

35 Why it is important? Patients with GCA can suffer ischaemic complications in non-cranial vascular territories. (18% aortic aneurysm or dissection, 13% largeartery stenosis). TAB is not diagnostic in extracranial GCA. Investigations with imaging techniques are warranted and consider treatment using systemic vasculitis protocols- More intensive and prolong treatment regime. Patients should have a chest radiograph every 2 years to monitor for aortic aneurysm. If large-vessel involvement is suspected, this may need supplementation with echocardiography or other imaging.

36 FDG-PET in Vasculitis Activated leucocytes overexpress glucose transporters and accumulate glucose and fluorine -18 fluorodeoxy glucose (FDG). Used in imaging LVV-GCA, Takayasu s, aortitis and chronic peri-aortitis. A Study demonstrated inflammation of the aorta and its branches in 92% of patients with PMR. Tracer uptake co-related strongly with CRP and ESR. FDG-PET may have a promising role in assessing disease activity and extent in non cranial GCA. Described vascular FDG uptake at diagnosis, in 29 (83%) of 35 patients with GCA. FDG uptake in the shoulders at diagnosis correlated significantly with the presence of PMR (P = 0.005). Vascular uptake decreased with treatment Large-vessel GCA. -Ann Rheum Dis 2004;63(7):870-3.

37 FDG-PET in Vasculitis Results using FDG-PET in vasculitis suggest the presence of subclinical inflammation of the great vessels in some patients with cranialgca or PMR. Direct evaluation of the temporal arteries is not possible with the whole-body PET techniques because of high uptake of FDG in the brain, because of the small diameter of the temporal artery. Can identify active vs inactive disease hence help in guiding management. Availability?

38 Role of PET in Large vessel vasculitis When typical clinical features are absent. When TAB is negative. Systemic symptoms predominate.(can exclude malignancies) Persistently high inflammatory markers. Poor response to steroids and other immunosuppressants.

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42 Color Doppler ultrasound in GCA Abnormalities reported in GCA patients include stenoses, occlusions, and the presence of a hypoechoic halo (halo sign) around involved arteries.

43 Color Doppler ultrasound in GCA Sensitivity and Specificity respectively were as follows: o Halo sign -periluminal hypoechogenic halo reflecting arterial wall edema 69 and 82 percent o Stenosis or occlusion 68 and 77 percent o Halo, stenosis, or occlusion 88 and 78 percent

44 Doppler ultrasound and giant cell arteritis Ana Marina Suelves, 1 Enrique España-Gregori, 1,2 Jose Tembl, 3 Stephanie Rohrweck, 1 Jose Maria Millán, 4 and Manuel Díaz-Llopis 1,4,5 Thus, CDU may be useful in application to the initial diagnosis and follow-up of patients with GCA, in view of its non invasive nature, reproducibility, and 100% correlation to the TAB findings in our series Ultrasound Better Than Biopsy in Giant Cell Arteritis. European League Against Rheumatism (EULAR) Congress 2014: Abstract OP0056. Presented June 12, 2014 June 14, 2014 The 87 patients with suspected giant cell arteritis underwent cranial ultrasound from January 2005 to July At 3-month follow-up, 36 patients had a confirmed diagnosis. Of the 30 patients with positive results on cranial ultrasound, 29 had a confirmed diagnosis of arteritis at 3 months.

45 Color Doppler ultrasound in GCA Still has not replaced TAB as the gold standard for diagnosis. But useful in.. When TAB is negative but clinical suspicion is high. When patient refuses TAB. To assess response to treatment. Identify relapses.

46 Summary Think Extracranial GCA in sub-optimally controlled or relapsing patients with polymyalgia rheumatica or cranial GCA. Large vessel involvement in GCA and PMR is becoming increasingly identified. PET helpful in LVV diagnosis particularly when systemic symptoms predominate and raised ESR or CRP. Large vessel vasculitis requires more intensive treatment and regular surveillance foe complications.

47 Thank you!