chronic inflammatory rheumatism associated with takayasu disease

6
Case Reports Chronic Inflammatory Rheumatism Associated With Takayasu Disease Kaouther Ben Abdelghani, 2 Alia Fazaa, 2 Khaoula Ben Abdelghani, 1 Ahmed Laatar, 2 Adel Khedher, 1 and Leith Zakraoui, 2 Tunis, Tunisia Takayasu disease is rarely associated with other autoimmune diseases. Therefore, the cases discussued herein are uncommon because we are reporting Takayasu disease associated with rheumatoid polyarthritis and spondylarthropathy. The first case concerns a 40-year-old woman presenting with Takayasu disease 11 years after the diagnosis of erosive and seroneg- ative rheumatoid polyarthritis. The upper limb arteries and 1 lower limb artery were affected. The second 41-year-old case presented with ankylosing spondylitis that had been evolving for 10 years. Human leukocyte antigeneB27 typing was negative. Takayasu disease was revealed by severe high blood pressure. In both cases, radiologic examination revealed a typical aspect of the aorta and its main collaterals. Rarely in the literature have these associations been re- ported, and the pathology remains unknown. INTRODUCTION First described in 1905, Takayasu disease is an inflammatory, obliterative, segmental arteritis of unknown etiology that affects the aorta and its main branches. It is characterized by the progressive development of stenoses, occlusions, or artery aneurysms that have different clinical manifestations. It was described in association with various auto- immune or inflammatory diseases. Association with rheumatoid polyarthritis (RP) or ankylosing spon- dylitis (ASP) has been rarely reported. Our 2 case reports are discussed herein, as is an overview of the published literature. METHODS Case 1 In 1991, our first patient, a 40-year-old woman, presented with chronic polyarthritis affecting the wrists and meta- carpophalangeal joints bilaterally and symmetrically, with morning stiffness lasting for >1 hour. Biology revealed an inflammatory syndrome. Testing for rheuma- toid factor (RF) and antinuclear antibodies (ANAs) was negative. Radiographic examination of the hands revealed geodic carpitis and bilateral radiocarpal pinching. Erosive and seronegative RP was diagnosed because the patient met 5 criteria for rheumatic disease as proposed by the American College of Rheumatology (ACR). She was initially treated with D-penicillamine (250 mg/day), prednisone (10 mg/day), and indometacine (100 mg/day). In 1999, bilateral coxitis required double total hip prostheses. Methotrexate (10 mg/week) and corticosteroids (5 mg/day) were jointly administered. In 2002, dyspnea on exertion, headaches, and intermittent claudication on the upper limbs appeared. A physical examination revealed the bilateral absence of pulses at the radial and humeral levels and at the right foot level. Blood pressure at the left lower limb level was normal. There was no carotidodynia and no cardiac murmur. Osteoarticular examination revealed a swan neck defor- mity of the fingers and ankylosis of the wrists. Articular count was 4/2. The disease activity score-28 (DAS-28) was 3.2. Pulmonary, neurologic, ophthalmic, and 1 Internal Medicine Init, Charles Nicolles Hospital, Tunis University, El Manar, Tunis, Tunisia. 2 Department of Rheumatology, Mongi Slim Hospital, La Marsae Tunis University, El Manar, Tunis, Tunisia. Correspondence to: Kaouther Ben Abdelghani, MD, Department of Rheumatology, Mongi Slim Hospital La Marsa, Tunis University, El Manar, Tunis, Tunisia; E-mail: [email protected] Ann Vasc Surg 2013; 27: 353.e1e353.e6 http://dx.doi.org/10.1016/j.avsg.2011.11.048 Ó 2013 Elsevier Inc. All rights reserved. Manuscript received: October 9, 2011; manuscript accepted: November 25, 2011. 353.e1

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Case Reports

1Internal MEl Manar, Tun

2DepartmeTunis Univers

CorrespondRheumatologyManar, Tunis,

Ann Vasc Surghttp://dx.doi.or� 2013 Elsevi

Manuscript re

25, 2011.

Chronic Inflammatory RheumatismAssociated With Takayasu Disease

Kaouther Ben Abdelghani,2 Alia Fazaa,2 Khaoula Ben Abdelghani,1 Ahmed Laatar,2

Adel Khedher,1 and Leith Zakraoui,2 Tunis, Tunisia

Takayasu disease is rarely associated with other autoimmune diseases. Therefore, the casesdiscussued herein are uncommon because we are reporting Takayasu disease associatedwith rheumatoid polyarthritis and spondylarthropathy. The first case concerns a 40-year-oldwoman presenting with Takayasu disease 11 years after the diagnosis of erosive and seroneg-ative rheumatoid polyarthritis. The upper limb arteries and 1 lower limb artery were affected. Thesecond 41-year-old case presented with ankylosing spondylitis that had been evolving for 10years. Human leukocyte antigeneB27 typing was negative. Takayasu disease was revealedby severe high blood pressure. In both cases, radiologic examination revealed a typical aspectof the aorta and its main collaterals. Rarely in the literature have these associations been re-ported, and the pathology remains unknown.

INTRODUCTION

First described in 1905, Takayasu disease is an

inflammatory, obliterative, segmental arteritis of

unknown etiology that affects the aorta and its

main branches. It is characterized by the

progressive development of stenoses, occlusions,

or artery aneurysms that have different clinical

manifestations.

It was described in association with various auto-

immune or inflammatory diseases. Association with

rheumatoid polyarthritis (RP) or ankylosing spon-

dylitis (ASP) has been rarely reported. Our 2 case

reports are discussed herein, as is an overview of

the published literature.

edicine Init, Charles Nicolles Hospital, Tunis University,is, Tunisia.

nt of Rheumatology, Mongi Slim Hospital, La Marsaeity, El Manar, Tunis, Tunisia.

ence to: Kaouther Ben Abdelghani, MD, Department of, Mongi Slim Hospital La Marsa, Tunis University, ElTunisia; E-mail: [email protected]

2013; 27: 353.e1e353.e6g/10.1016/j.avsg.2011.11.048er Inc. All rights reserved.

ceived: October 9, 2011; manuscript accepted: November

METHODS

Case 1

In 1991, our first patient, a 40-year-old woman, presented

with chronic polyarthritis affecting the wrists and meta-

carpophalangeal joints bilaterally and symmetrically,

with morning stiffness lasting for >1 hour. Biology

revealed an inflammatory syndrome. Testing for rheuma-

toid factor (RF) and antinuclear antibodies (ANAs) was

negative. Radiographic examination of the hands revealed

geodic carpitis and bilateral radiocarpal pinching. Erosive

and seronegative RP was diagnosed because the patient

met 5 criteria for rheumatic disease as proposed by the

American College of Rheumatology (ACR).

She was initially treated with D-penicillamine (250

mg/day), prednisone (10 mg/day), and indometacine

(100 mg/day). In 1999, bilateral coxitis required double

total hip prostheses. Methotrexate (10 mg/week) and

corticosteroids (5 mg/day) were jointly administered. In

2002, dyspnea on exertion, headaches, and intermittent

claudication on the upper limbs appeared. A physical

examination revealed the bilateral absence of pulses at

the radial and humeral levels and at the right foot level.

Blood pressure at the left lower limb level was normal.

There was no carotidodynia and no cardiac murmur.

Osteoarticular examination revealed a swan neck defor-

mity of the fingers and ankylosis of the wrists. Articular

count was 4/2. The disease activity score-28 (DAS-28)

was 3.2. Pulmonary, neurologic, ophthalmic, and

353.e1

Fig. 1. Thoracic angiotomodensitometry. Note the

regular circumferential parietal thickening concerning

the aorta arch.

Fig. 2. Thoracic angiotomodensitometry. Note the

regular circumferential parietal thickening concerning

the left subclavian artery at its origin.

353.e2 Case reports Annals of Vascular Surgery

dermatologic examinations were normal. ANA, antiphos-

pholipid antibodies, and anticoagulant circulating lupus

tests were negative.

A Duplex scan of supra-aortic trunks revealed circum-

ferential thickening of the left primitive carotid artery

wall, spreading to its bifurcation, and a tight and spreading

stenosis of the pre- and postvertebral left subclavian

artery. Right internal, external, and primitive carotid

arteries were patent.

Arteriography revealed a left subclavian occlusion

from its origin with the presence of a replacement

network of poor quality originating from the left primitive

carotid and intercostal arteries. It also revealed a long and

very tight stenosis of the right subclavian artery in its post-

vertebral part. Cardiac ultrasound revealed a thickening of

the descending thoracic aorta wall and an aortic and

mitral valvulitis. Therefore, Takayasu disease stage II,

according to the Moriwaki classification,1 was diagnosed

in association with deforming, erosive, and seronegative

RP. Corticosteroid therapy was increased to 1 mg/kg/day

for 1 month, with a progressive decrease over 10 months.

Ten years later, RP was stabilized. The patient no longer

presented with arthralgia or nocturnal awakening. Artic-

ular count equaled 0/0, the erythrocyte sedimentation

rate (ESR) was 7 mm, and C-reactive protein was nega-

tive. The DAS-28 was 2. Moreover, Takayasu disease

was in remission, with no systemic symptoms, no aggrava-

tion of ischemic symptoms, and no ESR acceleration. The

most recent explorations revealed no aggravation and no

new arterial lesions.

Case 2

A 41-year-old man presented with no medical history. At

17 years of age, the patient relates this history, he had

inflammatory back pain associated with inflammatory

arthralgia in the shoulders and wrists that generated

morning stiffness lasting for about 30 minutes; he also

had bilateral buttock pain. Arthritis in the wrists, left

elbow, and knees, with stiffness in the lumbar spine, was

also present. Cardiovascular, pulmonary, neurologic,

abdominal, and ophthalmic examinations were normal.

The ESR was 38 mm; lipid electrophoresis revealed poly-

clonal hypergammaglobulinemia. ANA and RFwere nega-

tive. A radiographic examination of the pelvis revealed

bilateral sacroillitis. Cervical spine radiography revealed

posterior joint ankylosis.

A diagnosis of ASP in its axial and peripheral form was

accepted and the patient was given phenylbutazone and

then indometacin. The evolution was good, with disap-

pearance of pains and arthritis. We then lost the patient

to follow-up.

Ten years later, in 1997, the same patient presented

with severe symptomatic high blood pressure and left

heart failure. A clinical examination revealed a murmur

along the subclavian arteries with an absence of pulse in

the upper limbs and systolic blood pressure (SBP) at 200

mm Hg at the level of the lower limbs.

A Duplex scan of the supra-aortic trunk revealed

a thickening of the arterial brachiocephalic trunk, the

subclavian, and the primitive carotid arteries wall,

without significant stenosis. Duplex scans of the upper

limbs revealed a tight bilateral subclavianeaxillarystenosis; a Duplex scan of the lower limbs was normal.

Arteriography revealed that the aortic arch was normal

but there was also a minimal stenosis at the left primitive

carotid artery origin and at its bifurcation, a stenosis at the

origin of the left vertebral artery, a bilateral subclavian

occlusion, and a tight ostial stenosis of the right renal

artery. Coronarography was normal.

Human leukocyte antigen (HLA) typing was A33 B8

type. Takayasu arteritis was diagnosed (4 criteria accord-

ing to the 1990 ACR). The patient was treated with corti-

costeroids and antihypertensive medications. Thereafter,

several complications occurred. First, a tight stenosis of

the right renal artery with renal ischemia required

Table I. Observations of associations between rheumatoid polyarthritis and Takayasu disease

Case no. Authors Age (yrs) Sex

Age at PRdiagnosis(yrs)

Age at Takayasudisease diagnosis(yrs) RF

Vascular lesionclassification(arteriographyor autopsy)

1 Korkmaz et al.6 36 F 34 36 + I

2 Sandring and Weil7 63 F 40 63 + I

3 Sandring and Weil7 65 F 55 65 + I

4 Falicov and Cooney8 16 F 16 20 e I

5 Reimer et al.9 49 F 44 49 + III

6 Rush et al.10 37 F 37 26 + III

7 Sketcher et al.11 53 F 50 53 e I

8 Mimura et al.12 50 F 48 50 + III

9 Gravellese et al.13 61 F 50 61 + I

10 Gravellese et al.13 82 F 74 82 e I

11 Gravellese et al.13 68 M 47 68 + III

12 Gravellese et al.13 61 F 58 61 + I

13 Gravellese et al.13 52 F 26 52 + I

14 Gravellese et al.13 69 M 68 69 + Aortic root

15 Gravellese et al.13 60 M 48 60 + ?

16 Gravellese et al.13 46 M 37 46 + II

17 Gravellese et al.13 67 M 65 67 + III

18 Gravellese et al.13 64 F 61 64 + III

19 Towned et al.14 44 M ? 44 + Aortic root

20 Nakabayashi et al.15 64 F 60 64 + III

21 Our case 40 F 20 28 e II

F, female; M, male; RF, rheumatoid factor; RP, rheumatoid polyarthritis.

Vol. 27, No. 3, April 2013 Case reports 353.e3

nephrectomy in 1999. Second, aortic insufficiency

required aortic valve replacement in 2003. Both diseases

remained stable with antihypertensive medicines, antico-

agulants (acenocoumarol), corticosteroids (prednisone

7.5 mg/d), and sulfasalazine (2 g/d) until 2010.

The patient was then hospitalized for recurrent arthral-

gias with asthenia. SBP was 150 mm Hg at the level of the

lower limbs. Except for humeral and radial pulses, all

pulses were present. Dorsal kyphosis and stiffness of the

whole rachis (Schober index, 0; occiputewall distance,

6 cm). The ESR was high (60 mm) and CRP was 10 mg/L.

Echography of the supra-aortic trunks revealed a mild

infiltration. Angiotomodensitometry revealed regular and

circumferential parietal thickening of almost the entire

aorta, with a slight aneurysmal dilatation of the aortic

arch (Fig. 1), a regular and circumferential parietal thick-

ening of the left subclavian artery at its origin (Fig. 2), an

aneurysmal dilatation of the left pulmonary artery trunk

(lobar and segmental branches), and a superior mesen-

teric artery occlusion taken up by collateral arteries. The

left renal artery with a normal caliber was patent. The

patient’s symptoms of ASP have become more severe

(Bath Ankylosing Spondylitis Functional Index [BASFI],

2.9; Bath Ankylosing Spondylitis Disease Activity Index

[BASDAI], 4.8), and because the inflammatory biologic

syndrome and arterial parietal thickening featured the

activity of both diseases, an antietumor necrosis factor

a (anti-TNFa) treatment was recommended but has not

been carried out yet.

DISCUSSION

According to the 1990 ACR criteria,2 Takayasu

disease was diagnosed in our patients. Typically,

this disease has 2 evolutive phases. A preocclusive

phase is characterized by general symptomatology,

then an arterial occlusion phase is marked by

a lack of pulses and polymorphic manifestations

linked to ischemia. The diagnosis was given at this

second stage in both of our patients. An association

probably not coincidental with different autoim-

mune or inflammatory diseases had already been re-

ported, the association with the Crohn’s disease3,4

being the less rare.

Arthralgias and arthritis are frequent during the

initial stage of Takayasu disease. They were reported

by Hall et al.5 in 56% of cases. Our observations are

different because Takayasu disease occurred along

with rheumatic chronic inflammatory pathologies

in both cases (RP and ASP, respectively).

The literature review included only a few similar

cases. If aortitis can complicate RP evolution, only

20 patients presentingwith RP and Takayasu disease

have been reported until 2001 (Table I).6e15 The

analysis of these cases shows a female predomi-

nance (14 women; 6 men). On average, RP

appeared at 48 years of age and Takayasu disease

Table II. Observations of associations between ankylosing spondylitis and Takayasu disease

Case no. Authors SexAge(yrs) Arterial lesions Joint lesion

ESR(mm/hr)

Delay betweenASP and Takayasudisease (yrs)

1 Paloheimo

et al.17M 46 Subclavian, L carotid,

vertebral, and renal

SI, lumbar S, and

peripheral J

67 4

2 Paloheimo

et al.17F 24 R subclavian SI 112 3

3 Paloheimo

et al.17F 25 Subclavian and carotid SI and lumbar S 20e120 4

4 Paloheimo

et al.17F 24 Subclavian, L vertebral,

and carotid

SI and lumbar S 74 d

5 Ghozlan

et al.18F 22 L subclavian,

L vertebral, L carotid,

and L renal

SI and knees 30 3

6 Hull et al.19 F 63 Subclavian and

vertebral

SI, lumbar S, and

peripheral J

115 3

7 Magaro

et al.20F 17 Subclavian and L renal SI and lumbar and

thoracic S

86 Concomitant

8 Cherin

et al.21F 24 R carotid, subclavian,

ABCT, vertebral, and

axillary

SI and peripheral J 106 1

9 Cherin

et al.21M 55 Carotid, subclavian,

and vertebral

SI and peripheral J 130 26

10 Cherin

et al.21F 22 Subclavian, ABCT,

L vertebral, and

R pulmonary

SI and knees 70 18

11 Soubrier

et al.22F 55 Subclavian and

L superficial femoral

SI, heel pain, and

R knee

100 Concomitant

12 Schuetz

et al.23M 45 Type III Lumbar and

thoracic S

48 Previous arteritis

13 Dziadzio

et al.24F 17 ABCT, carotid, and

L subclavian

SI and peripheral J 57 2

14 Acar et al.25 F 14 Carotids, R renal,

superficial femoral,

and superior

mesenteric

SI 66 Concomitant

15 Taharboucht

et al.26F 26 L subclavian, humeral,

and thoracic aorta

SI and cervical and

lumbar S

65 9

16 Our case M 41 L carotid, G vertebral,

subclavian, and

R renal

SI, cervical S, and

peripheral J

38 10

ABCT, arterial brachiocephalic trunk; ESR, erythrocyte sedimentation rate; F, female; J, joint; L, left; M, male; R, right; S, spine; SI,

sacroillitis.

353.e4 Case reports Annals of Vascular Surgery

was diagnosed at 55.5 years of age. As in our case,

the events linked to Takayasu disease have appeared

secondarily to those of RP in most cases (18 cases).

The time was variable, reaching or exceeding 10

years for 6 cases, as in our patient.

Clinical, evolutive, and epidemiologic specific-

ities do not seem to exist when these 2 diseases are

associateddexcept for Takayasu disease, where

the mean age of occurrence is higher than usual.6

In 3 cases, it was a seronegative RP. The prognosis

is still dominated by vascular lesions, and the 7

deaths registered in the literature were related to

Takayasu disease.We cannot exclude amere coinci-

dence in the coexistence of Takayasu disease with

a RP. In our observation, the long delay between

RP and Takayasu disease is coincidental.

However, the number of observations illustrating

such an association or a coexistence with other

autoimmune diseases4 is not caused by chance

along. Moreover, immunologic abnormalities play

an important role in both Takayasu disease and

RP. The possibility of an immunologic reaction

that is common to both pathologies, with genetic

predisposition, could be discussed.4

Vol. 27, No. 3, April 2013 Case reports 353.e5

ASP corresponds to inflammatory arthritis of the

spine and sacroiliac joints of unknown origin. It

mainly affects young men, with a sex ratio of 2:1.

Most of these patients are HLA-B27epositive. It

also includes different visceral lesions: cardiac,

pulmonary, digestive, ophthalmic, neurologic, and

dermatologic. The most frequent cardiovascular

lesion corresponds to aortitis of the proximal part

of the aorta with, as in our patient, valvular aortic

insufficiency requiring aortic valve replacement.

Lassalle et al.16 have performed a recent review of

aortitis during ASP.

Patients presenting with the coexistence of

authentic Takayasu disease are much rarer. The first

4 cases were described in 1966 by Paloheimo et al.17

A total of some 15 observations has been identified

up to 2010 (Table II).17e26 Their analysis reveals

that women are more commonly affected, with an

age of onset ranging from 14 to 61 years. HLA-B27

positivity is nonconstant and less frequent than in

isolated ASP. Our patient was HLA-B27enegative.

As in our case, ASP is usually diagnosed before

Takayasu disease. This association is rare, but some

arguments are in favor of its noncoincidental char-

acter. Women are disproportionately affected,

which is in contrast to the male majority usually

observed in ASP. Moreover, ASP and Takayasu

arteritis are TH1 cellemediated. TH1 cytokines may

play an important role in the common pathogenesis

of these diseases.27 One of the preferential patho-

logic associations with Takayasu disease is Crohn’s

disease, the joint manifestations of which belong

to the same spondylarthropathy group.

On the other hand, common pathogenic mecha-

nisms, supported by antigenic analogy between the

aorta and the entheses, could favor the occurrence

of these 2 diseases. Takayasu disease occurrence

during these 2 rheumatologic diseases of different

nature could be explained by the following hypoth-

esis: Takayasu disease could be based on immuno-

logic abnormalities caused by previous different

diseases (either infectious or autoimmune, such as

RP, or of other unknown origin, such as ASP).4

Therefore, as reported by Ohta et al.,4 out of 36

patients with Takayasu disease, 11 (30%) presented

with at least 1 another chronic or subacute inflam-

matory pathology. However, up to now, no partic-

ular favorable genetic conditions have been

identified.

Therapeutic implications exist when Takayasu

disease is diagnosed along with chronic rheumato-

logic pathology. They include medical means and

revascularization procedures. First-line treatment

consists of introducing or increasing general cortico-

steroid therapy. However, it has only a symptomatic

efficacy on the different systemic manifestations of

Takayasu disease. It was successful in our first

patient. In case of failure, methotrexate, cyclophos-

phamide, or mycophenolate mofetil can be

prescribed, without preference for any single drug

among them. Yokoe et al.,28 in their observation of

RP associated with Takayasu disease, underline the

importance of associating tacrolimus immosuppres-

sor treatment, which could be effective on the

2 pathologies. Anti-TNFa can be prescribed as a ther-

apeutic alternative in corticosteroid-resistant forms

of Takayasu disease29 or in nonsteroidal anti-

inflammatoryeresistant forms of ASP.30,31 The effi-

cacy of this treatment was also reported in Takayasu

disease associated with Crohn’s disease.32 There-

fore, anti-TNFamedications are an efficient therapy

for the second patient. Besides, different interven-

tional vascular techniques can treat arterial stenosis

with more or less durable long-term results.33

Medical treatment is systematically associated and

its indications depend on the arteritis impact: percu-

taneous angioplasty, with or without stent, endar-

terectomy, or bypass of the arterial brachiocephalic

trunk. As for our second patient, in cases of severe

aortic insufficiency, aortic valve replacement is

necessary.

CONCLUSION

Our 2 patients illustrate a rare association of 2

inflammatory pathologiesdrheumatologic and

vasculardwhose link has not yet been identified.

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