Clinical case 1

A 73-year-old male presents with a headache.

He has an atherosclerotic heart disease and is a former heavy smoker.

1. What further information is needed for the diagnosis?

Answer :
-The location, quality and severity of the headache?
- For how long has he been experimenting this symptom? Has he experienced these symptoms before? Is the pain
stable, intermittent or getting worse?
- Is the headache accompanied by neurological symptoms like dizziness, visual disturbances, paraesthesia or paralysis?
- Are there systemic symptoms like; fever, weight loss, muscle pain, skin rash or fatigue?
- Are there additional pain and/or stiffness in the neck, shoulders and hip-girdle area?
- Has the patient jaw claudication or scalp tenderness?
The patient reports that the he had a sudden onset of headache, as he woke up this morning. It is located all
over the head, but more intense over the right temporal region. The headache is continuously present.
There were no accompanying symptoms from the neck, shoulders or hip-girdle. He does not report; fever, weight loss,
visual disturbances, neurological symptoms, jaw or chest pain.
The blood pressure was 134/86 mmHg.
Blood test results: ERS 3 mm/h and C reactive protein was 3 mg/L.
2. Which of the following diagnosis should you consider as differential diagnosis?
a. Cerebrovascular incident
False. Probably not. Headache can be a presenting symptom of cerebrovascular incident. But you would expect
additional symptoms two
weeks after the onset of the headache.
b. Migraine
False. Probably not. The headache is a new symptom and is continuously present without aura.
c. Giant cell arteritis
True. Should be considered. Although the inflammatory parameters are normal this could be an early stage of the GCA.
At this point he has no typical accompanying symptoms that clearly points you in the direction of giant cell arteritis.
d. Sinusitis
True. Should be considered
e. Brain tumour
True. Should be considered. A new unexplained headache can be the presenting symptom of a brain tumour. The
headache is often worse in the morning. In this case the headache is continuously present with no variations of the
intensity throughout the day.
The patient was admitted to a MRI scan of the head that showed no signs of bleeding, cerebral ischaemia or brain
tumour. Chronic age related changes commonly seen in his age were described.
He was further examined by an otolaryngologist, that revealed no signs of sinusitis or infection.
After four weeks from the onset of headache he experienced jaw claudication, and pain in the area around the left ear.
He also experienced scalp tenderness.
He did not suffer from visual disturbances.
New blood test showed an increase in C-reactive protein to 55, ESR was still within the normal range but had increased

from 3 mm/h to 15 mm/h

3. What diagnosis do you now suspect?

Answer :
a) Cerebrovascular incident
b) Migraine
c) Giant cell arteritis(GCA)
d) Sinusitis
e) Brain tumour
Giant cell arteritis is a common disease of the geriatric age group in the western Europa especially. Headache is the
most common symptom of GCA (72%) which is often located at temple and occipital region. Jaw claudication is a
clinical symptom that has a high specificity of GCA, but a low sensitivity.
The superficial temporal artery (STA), occipital, posterio auricular or facial arteries demonstrate thickening, nodularity,
tenderness or erythema.
Laboratory findings included elevated C reactive protein. The ESR was still within the normal range, however, the ESR
may be normal in 22.5 % of biopsy proven GCA cases.

4. How would you examine the patient further to confirm your diagnosis?

Answer :
Harvesting a sufficient segment of STA (commonly more than 2 cm) and subjecting the artery to meticulous histological
examination remains the gold standard of diagnosis. The histopathological changes in the temporal arteries include
luminal stenosis, intimal proliferation and disruption of internal elastic lamina by a mononuclear cell infiltrate. The
involvement is typically patchy (skip lesions).
Duplex sonography of the temporal arteries, with demonstration of a dark halo around the artery, is emerging as a viable
alternative and is a non-invasive procedure. It has a reported sensitivity of 73 % and a high specificity. The
demonstration of bilateral dark halos seems to correlate well with development of ischemic complications in GCA, and
has in experienced hands, a good correlation with biopsy proven GCA.
You perform a duplex sonography of the temporal arteries where you are able to demonstrate a dark halo around the
temporal arteries on both sides, the halo is more protruding around the left temporal artery.
A biopsy from the left temporal artery is harvested, the size of the segment from the artery is 13 mm.
The segment is examined by a pathologist who describes the findings as a muscular artery with no apparent signs of
inflammation.
In this case you have a 73-year old male with typical symptoms of giant cell arteritis. Duplex sonography was positive
with a characteristic halo around the temporal arteries, while the harvested but rather short biopsy was considered
negative.
5. Would you harvest a second biopsy from the right temporal artery in order to try to confirm the diagnosis of
GCA?

Discuss :
See figure 1 - Summarizes the approach to patients with suspected GCA
The inflammatory lesions in GCA is often patchy (skip lesions). Therefore, in case of negative biopsy, a
repeated biopsy from the contralateral temporal artery, may secure the diagnosis if positive for CGA, but we could also
risk to have another false positive biopsy.
Another issue is that, not all patients are motivated to have a contralateral temporal artery biopsy.
Since up to 60% of patients with GCA may demonstrate involvement of aorta and its branches, imaging studies (CT
angiography, MRA or 18F-FDG-PET-CT) should be considered.
Patients of suspected GCA, who have typical symptoms of GCA, but are biopsy negative, are described to have less

constitutional symptoms, less arterial wall abnormality and have lower chances of ischemic complications, as compared
to biopsy positive cases.
Clinical case 2
A 32 year old man presents with a 6 month history of back and neck pain.

1. What should be the next questions in the evaluation of this patient?

Answer : You should try to better define the patient's back pain. Did it begin insidiously? Does it wake him during the
night? When is it worse - At the end of the day or in the morning? Does it improve with physical activity? Is it
accompanied by morning stiffness? The goal is to establish whether the patient suffers from inflammatory or mechanical
back pain
The patient reports that his back pain is most prominent in the morning and awakens him during the second half of the
night. In addition, he reports pain and swelling in his ankle and knee. On physical examination, there is synovitis of the
right ankle and knee as well as a positive Patrick sign. Likewise, psoriatic plaques are present in the scalp.
2. To what broad group of rheumatological syndromes does this patient belong?

Answer : Articular pattern

3. Within the articular conditions, which one of the following diagnosis is the most probable?
a. Osteoarthritis
False. Osteoarthritis does not commonly target the ankle or wrist and synovitis is not a feature. Furthermore this patient
is very young to get osteoarthritis in the absence of prior trauma
b. Rheumatoid Arthritis
False. The presence of axial symptoms and the isolated involvement of large joints are not typical of rheumatoid
arthritis, which usually involve multiple small and large joints, upper and lower limbs, and reasonable symmetry of
involvement.
c. Spondyloarthritis
True. The presence of inflammatory axial symptoms and the involvement of just a few large joints are typical of
spondyloarthritis.
d. Gout
False. Gout usually causes acute self-limiting episodes of florid synovitis with common targeting of the first
metatarso-phalangeal joint. Inflammatory spinal symptoms are not a feature.

4. Which one of the following features is associated with spondyloarthritis ?

a. Retinitis
False. Retinitis can be associated with vasculitis or Behet's syndrome but not with spondyloarthritis.
b. Episcleritis
False. Episcleritis associates with conditions that cause vasculitis, for example rheumatoid arthritis and systemic
vasculitides.

c. Uveitis
True. Anterior uveitis (iritis) associates with spondyloarthritis.
d. Dry eyes
False. Scleritis associates with conditions that cause vasculitis, for example rheumatoid arthritis and systemic
vasculitides.
e. Ischemic optic neuritis
False. Ischemic optic neuritis is typical of temporal arteritis.
The patient undergoes an X-ray of the sacroiliac joints which is interpreted as normal. HLA-B27 is negative. The ESR
and CRP are increased. RF and ANA are negative.
5. What is the most probable diagnosis?
a. Behets disease
False. Although Behets disease was previously considered within the seronegative spondyloarthritis group, this
patient has no orogenital ulcers, skin or eye lesions which are the commonest manifestations of Behets.
b. Ankylosing spondylitis
False. The absence of sacroiliitis in the X rays of the SIJ rules out the diagnosis of ankylosing spondylitis according
to the modified New York criteria.
c. Reactive arthritis
False. Although reactive arthritis is part of the spondyloarthritis spectrum, the absence of a triggering event and the
duration of symptoms do not support the diagnosis.
d. Psoriatic arthritis
True. This patient suffers from inflammatory back pain and oligoarthritis and is RF negative. The presence of axial
symptoms and the isolated involvement of large joints are not typical of rheumatoid arthritis. The absence of sacroiliitis
in the X rays of the SIJ rules out the diagnosis of ankylosing spondylitis according to the NY criteria. However, an
MRI examination of the sacroiliac joint would probably demonstrate active sacroiliitis. Although reactive arthritis is part of
the spondyloarthritis spectrum, the absence of a triggering event and the duration of symptoms do not support the
diagnosis. The combination of asymmetric oligoarthritis, psoriasis and RF negative is typical of psoriatic arthritis