Case Presentation

Case presentation

1. CASE PRESENTATION
By
Rasha Ali Abdel-Noor
Lecturer of internal medicine
&Rheumatology.
Tanta University, Faculty of
Medicine.

2. HISTORY
Afemale patient named F.E.E aged 47 years
from Elmehalla Elkobra ,Gharbia,married
and has 3 offsprings ,housewife and has no
special habits.
Complaints:
Acute sever pain in the 4 limbs mainly
in the lower limbs 2 days before
admission(Decemper 2013).

3. HISTORY OF THE PRESENT ILLNESS
 The condition has been started two days
before admission by acute onset of sever
pain in both lower limbs from toes till
knees extending to upper limbs and it was
associated by tingling and numbness
sensation it was not associated with fever
or any arthralgia or mucocutaneous rashes.
These symptoms occurred one week after
an attack of diarrhea for which she received
incomplete course of ciprofloxacine.

4. PAST HISTORY
No past history of diabetes or hypertension
or chronic disease of medical importance.
No history of drug use or drug allergy.

Family history:
 Irrelevant

Social history:
Average socioeconomic status.

5. EXAMINATION
General appearance:
The patient was fully conscious ,alert looks ill
with no special facial expression with normal
decubitus in bed and average body built.
Vital signs:
Pulse: absent radial , ulner and brachial
pulsations in both upper limbs.
absent both dorsalis pedis, ant and posterior
tibial arteries weak femoral pulsations.
Intact pulsations in both carotids about 80 b/m
regular rhythm with average force and
volume.

6. BP: undetected in 4 limbs.
RR: 20 c/m
Temp: 37 C
Head &neck:
Central trachea.
No enlarged thyroid gland.
 No congested neck viens.
No lymphadenopathy.

7. Chest : bilateral equal air entry with
normal vesicular breathing .
Heart: normal heart sounds ,no added
sounds or murmer.
Abdomen: lax abdomen ,no organomegaly
or ascites or bruit.
Extremities:
absent pulsations as described above.
Cold extremites ,poor capillary filling but
with no colour changes or any skin
lesions.

8. Musculoskeletal examination :
No joint swelling, tenderness or limitation
of movements
Neurological examination:
Motor power & deep reflexes were normal
Sensation : parathesia at 4 limbs

9. INVESTIGATIONS
Duplex study of 4 limbs:
Marked diffuse narrowing of arterial tree
of both lower limbs starting from external
iliac arteries with absent flow at both
anterior tibial and dorsalis pedis arteries
Marked diffuse narrowing of both brachial
arteries with absent flow in both radial
and ulner arteries.
Absent atherosclerotic changes.
Patent superfical and deep venous system.

10. INVESTIGATIONS

FBG:90mg /dl
BU: 70 mg/dl
SC: 1.1 mg /dl
CBC:
HB : 11 gm /dl
WBCs : 15000 /cmm
Platlets : 201,000/cmm

11. L.F.T:
Bilirubin : 1mg/l
SGPT: 76 u/l
SGOT: 93u/l
S. Albumin: 3.4 gm/l
S.globulin : 3.1
ESR: 45 1
st
hr ,65 2
nd
hr
CRP :-ve
Abd US : normal
ECG: Normal sinus rhythm.
Echo: Normal

12. Emperically and urgently we treated this
patient as a case of an acute & sever form
of a large vessel vasculitis ,so we started
Pulse steroid one gram /day for 3 days .
IV pentoxyfilline every 8 hours .
 Prophylactic dose of LMw heparin.
Low dose asprine.
And preparing her for CT angiography and
other immunological assay.

13. CT ANGIOGRAPHY
Normal aortic arch ,descending thoracic
and abdominal aorta with diffuse
narrowing of both external iliac arteries
with absence of atherosclerotic changes
favor the diagnosis of peripheral
vasculitis.

19. RESPONSE
Marked improvement of her ischemic pain
after the 2
nd
dose of pulse steroid ,but the
pulsations still was undetected.
With completing her lab . Investigations:
RF : -ve
ANA: -ve
P&C (ANCA): -ve
Virology:
HCV antibodies –ve
HBsAg -ve

20. HIV antibodies : positive

22. SURPRISE
Our patient is an HIV patient since 16
years and she is already on HAART
1-Aluvia:lopnavir200 mg &50 ritonavir
2-Aurobindo:emtricitabine 200 mg and
tenofovir disoproxil fumerate 300 mg.

She and her family denied all this
history due to its social stigmata.

23. DILEMMA
Is this case is large vessel vasculitis
associated with HIV or it is actually
HIVrelated vasculitis????

What about its treatment???

Is it a mistake to start with pulse steroid???

How could we continue??????

24. HIV RELATED VASCULOPATHY
Since the beginning of the HIV era in the
early 1980s much has been written about
rheumatic and immunologic complications
observed in this patient population,
including the co-occurrence of systemic
vascular inflammatory diseases .


Calabrase L H: Vasculitis and infection with the human immunodeficiency
virus.Rheum Dis Clin North Am 1991; 17: 131-47

26. TYPES
Category I refers primarily to vasculitides diagnosed
according to ACR classification schemes that have been
reported in the setting of HIV infection.
Category II refers to secondary forms of vasculitis (e.g.
where a precipitin or mechanism can be identified such
as a specific infection or drug induced hypersensitivity.
 Category III describes those vascular disorders that
appear to be unique and for which strong consideration
should be given.
FA L K RJ, A N D R A S S Y K e t al.: Nomenclature of systemic vasculitides.Proposal of an international
consensus conference.Arthritis Rheum 1994; 37: 187-92.
HU N D E R GG, A R E N D W P, B L O C H D A e tal.: The American College of Rheumatology
1990 criteria for the classification of vasculitis.Introduction. Arthritis Rheum 1990; 33:1065-7.

27. CATEGORY I PRIMARY VASCULITIS IN HIV
HIV has infected an estimated 75
million people worldwide over the past
25 years , it should not be surprising to
find documented accounts of just
about any form of vascular
inflammatory disease reported on a
chance association basis.


UNAIDS/WHO: AIDS epidemic update. December 2000: 1-28.

29. Miscellaneous disorders with vascular
inflammation as a feature reported in HIV
Cutaneous PAN
Behcet’s syndrome
Primary angiitis of the central nervous system
Erythema nodosum.
 Rheumatic fever ,Erythema elivatinum
diutinum.
 Degos disease
Angiocentric lymphoproliferative disorders
Coronary arteritis
Eaosinophilic vasculitis
Leukocytoclastic vasculitis with follicular
accentuation

30. PATHOGENESIS
The pathophysiology of these reported cases is
also unclear but may be due to direct or an
indirect role for HIV itself. by way of uncontrolled
immune activation with attendant cytokine
release , which characterizes the underlying
infection.


L.H. Calabrese Infection with the Human Immunodeficiency Virus type 1 and
vascular inflammatory disease Clin Exp Rheumatol 2004; 22 (Suppl. 36):S87-S93.

31. CATEGORY II SECONDERY VASCULITIS
Given the immunopathology of progressive
immunodeficiency and opportunistic infections. It
is well known that vasculitis may arise in the
course of virtually any type of infection
(i.e,bacterial, fungal, mycobacterial, viral
parasitic) and that such pathologic lesions may
come about by way of direct angio-invasion or via
aberrantly directed host-mediated immune
defenses.

32. Secondary forms of vasculitis.
Infectious arteritis.
Cytomegalovirus .
Pneumocytstis carinii .
Toxoplamosis .
Herpes zoster associated .
Hepatitis C associated .
Drug-induced vasculitis .
Vasculitis associated with antiretrovirals .

33. CATEGORY III VASCULAR INFLAMMATORY
DISORDER UNIQE TO HIV
(1) cerebral vasculopathy with aneursymal
dilatation in HIV infected children.
(2) large vessel disease in HIV infected
patients .




T V MULAUDZI Vasculopathy is a major feature of HIV disease. CME July 2009 Vol.27

35. Aneurysms

This disease affects much younger patients The
median age is between 30 and 40 years. However, the
majority of patients infected with HIV are females and
the reason for the male preponderance of aneurysmal
disease is unknown.
The pathogenesis of the aneurysms is uncertain.
Histology shows obliterative endarteritis involving
the vasa vasora of the major vessels. These vessels
are surrounded by neutrophils, which in turn are
surrounded by a cuff of plasma cells, lymphocytes and
monocytes. This eventually leads to thrombotic
occlusion of the vasa vasora with transmural necrosis
of the vessel wall, probably due to ischaemia.

36. The common sites for the aneurysms are
the common carotid and the superficial
femoral artery .They can, however, be
found throughout the body, tend to be
multiple, and typically have a multi-
loculated appearance . Many are in fact
false aneurysms due to disruption of the
vessel wall at the point of transmural
necrosis.

38. OCCLUSIVE DISEASE
HIV-associated arterial occlusive disease is recognised
as a specific clinical entity. As for aneurysmal disease,
young males with a median age of 30 - 40 years are
mainly affected; it is difficult to explain this male
preponderance.
The underlying cause of occlusive disease is thought to
be related to vasculitis. The histological findings are
similar to those found in HIV-associated aneurysmal
disease. It has been suggested that a hypercoagulable
state might be involved. There have been reported
findings of anti-phospholipid antibody syndrome,
deficiencies of free protein S, protein C and anti-
thrombin 3, but these have been sporadic reports.

39. Clinically most patients present with advanced
disease and critical ischaemia affecting the lower limb.
In most patients the disease is confined to one limb, for
reasons that are obscure. It is interesting to note that on
Duplex Doppler imaging there is what appears to be a
pathognomonic sign with hypoechoic spotting within the
arterial wall (string of pearls sign).

Mulaudzi TV, Robbs JV, Pillay W, et al. Thrombectomy in HIV related peripheral arterial thrombosis: a
preliminary report. Eur J Vasc Endovasc Surg 2005; 30(1): 102-106.
 Nair R, Robbs JV, Chetty R, Naidoo NG, Woolgar J. Occlusive arterial disease in HIV-infected patients: a
preliminary report. Eur J Vasc Endovasc Surg 2000; 20: 353-357.
 Botes K, Van Marle J. Surgical intervention for HIV-related vascular disease. Eur J Vasc Endovasc Surg
2007; 34: 390-396.

40. HIV& TAKAYASU
There is striking clinicopathologic overlap
withTakayasu's disease.
Features of similarity include :
the young age of the patients, presence of multiple
aneurysms, involvement of large vessels, arteritic
and postarteritic changes of the vasa vasora, a
temporal sequence of events (active and healing
stages), and the absence of an obvious causative
agent.

41. Differences from Takayasu's:
disease are the predominance of males, the
proliferation of slit-like vascular spaces in the
adventitia,the pronounced leukocytoclastic vasculitis
of the vasa vasora, the absence of prominent intimal
proliferation and atherosclerosis, and the clustering
of the cases that coincides with the HIV/AIDS
epidemic.
The overlap with Takayasu's disease is not
surprising given its so called idiopathic
etiopathogenesis and overlap with several
arteritides. In other words, there is a chance
association between H1Vand vasculitis.

RUN JAN CHETTY, SIXTO BATITANG AND RAJ NAIR, Large Artery Vasculopathy in HIV-Positive Patients:
Another Vasculitic Enigma HUM PATHOL 2000 31:374-379

43. TREATMENT
Therapy for HIV-associated vasculitis
remains controversial and problematic,
with the largest reported experience being
that of Guillevin and colleagues who
advocate a combination of glucocorticoids,
apheresis and antiviral therapy .


GISSELBRECHT M, COHEN P, LORTHOLARY O et al.: Human
immunodeficiency virus related vasculitis. Clinical presentation of and therapeutic
approach to eight cases. Ann Med Interne (Paris) 1998; 149: 398-405.

45. Standard treatment has been offered for aneurysms,
including prosthetic grafts, and relatively recently,
endovascular therapy has been performed with stent
occlusive disease
In 33 of 36 (71%) patients, salvage was attempted
comprising thrombectomy with or without thrombolysis;
bypass was attempted in the remaining three patients.
Of the 64 patients, who had chronic occlusive disease,
primary amputation was necessary in 30 (47%) while no
treatment was offered to nine (14%) who were deemed to
be pre terminal.
Twenty-one patients were offered bypass surgery, and
four an endovascular procedure

46. SO OUR DECISSION WAS:
To continue on 40 mg prednisolone
Oral pentoxyphylline
Low dose asprine
Follow up after two week:
She was very well no ischemic pain but only mild
neuropathic pain for which pregabalin was
prescribed.
Pulse was detected well in her RT radial ,ulner
and brachial also her left dorsalis pedis and ant
tibial.
Felt but weaker lt radial and ulner.
Still undetected in her RT dorsalis pedis.

47. NEW DOPPLER AFTER ONE MONTH
Patent venous and arterial system of both
upper and lower limbs with no plaques or
stenosis with only biphasic flow for follow
up.

52. So we started gradual withdrwal of
steroid dose 5mg every week till it
was completely stopped.
Patient is now on pregabalin only
and HAART.
Another doppler study was done
after steroid withdrwal and it
showed the same results.

53. CBC: ( 4-4-2014)
HB:12.6
Platlets:247,000
WBCs:5800
Immunophenotyping :
CD4 : 734 (38%) N(430-1600)
CD8 : 618 (32%) N(280-1100)
CD4/CD8 : 1.2 N(1.2-6.2)
Viral load: ??

54. DILEMMA
Is this case is large vessel vasculitis
associated with HIV or it is actually
HIVrelated vasculitis????

What about its treatment???

Is it a mistake to start with pulse steroid???

How could we continue??????

55. OUR CASE IS:





HIV RELATED LARGE VESSLE VASCULITIS
CATEGORY III