RHEUMATOID VASCULITIS AMONG NIGERIANS

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Feb 22, 2014 (3 years and 6 months ago)

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RHEUMATOID VASCULITIS AMONG NIGERIANS


BY




DR. S.A. OGUNTONA MBChB, FWACP. CERT. RHEUM.




DEPARTMENT OF MEDICINE


OLABISI ONABANJO UNIVERSITY/ OLABISI ONABANJO


UNIVERSITY TEACHING HOSPITAL.









SAGAMU. OGUN STATE.


NIGERIA.






CORRESPONDENCE TO :
-

DR. S.A. OGUNTONA

P.O.BOX 231, SAGAMU.

OGUN STATE. NIGERIA.

Mobile
-

08035534449

E
-
mail
-

oguntonasa@yahoo.com
,
oguntonasa@gmail.com














ABSTRACT


Background
-



Rheumatoid vasculitis

(RV) is an inflammatory condition of the small and medium

sized vessels that affects a subset of patients with established rheumatoid arthritis. It
has a fast array of clinical manifestations with a predilection for the skin, and the
peripheral nervous s
ystem. Diagnosis relies on exclusion of other causes with similar
lesions because of lack of specific signs and symptoms.


Methods
-



A
ll cases of rheumatoid
arthritis that met the 1987

American College of Rheumatology
criteria were enlisted. Twenty (20) cases were seen over three and a half years (July
2009

Dec. 2012). Rheu
matoid factor, anti
-
cyclic citri
llunated peptide
(CCP)
,
erythrocyte sedimentation rate (ESR), full blood count (FB
C) were determined. Plain
radiograph of the hands and feet were requested for where appropriate.


Results
-


All the patients had elevated ESR. Significant titre of rheumatoid factor was present in
eight (8) patients. There was positive anti
-
CCP in those w
ho are able to afford it.
Marked finger deformities were noticed in six of the patients, while only two patients had
significant feet deformity. One patient presented with multiple feet digit
s

loss

as a result
of infarction with subsequent surgical removal
.


Conclusion


Although, rheumatoid vasculitis is now a relatively rare complication of long standing
rheumatoid arthritis, possibly because of the improved management strategies. It still
remains an important complication of rheumatoid arthritis that need
s to be promptly
recognized and treated. This stands as the first case

of rheumatoid vasculitis

seen in
the clinical practice of the author.


Key words
-

rheumatoid vasculitis, rare, vessel inflammation, digital infarction.





INTRODUCTION

Rheumatoid
vasculitis (RV) typically affects small and medium
-
sized vessels
1,2
.
It is
associated with high rates of prematured mortality with up to 4
0% of patients dying by
five

years as well as significant morbidity due to both organ damage from vascultis

and
consequences of the treatment

3,
4
.

Rheumatoid vasculitis

usually occurs in patients who have longstanding disease,
generally of more than ten years duration
5
.

It usually associated with poor prognosis.
Clinical
l
y, patients have more s
e
vere rheumatoid
arthritis with destructive joint disease
and other features of extra
-
articular disease
6
.

The appearance of rheumatoid vasculitis

may be associated with a rise in acute
-
phase
markers, including the erythrocyte sedimentation rate and C
-
reactive protein, together
with thrombocytopenia and anemia of chronic disease
7,8
.

It is sometimes noted t
hat arthritis is

not active when the feature
s of systemic vasculitis
occur in patients who are thought to have “burned
-
out
-
disease”. More severe features of
rheumatoid vasculitis are frank infarction of the digits and mononeuritis multiplex

9,10,11
.

Laboratory tests may support, but do not confirm
a diag
nosis of rheumatoid vasculitis.
Discovering skin or nervous system abnormalities in a patient with extra
-
articular
manifestaions of rheumatoid arthritis and constitutional symptoms should raise a high
index of suspic
ion for rheumatoid vasculitis an
d
must prompt further evaluation.

Options for definitive diagnosis include biopsy of involved skin and muscle and

nerve
biopsy of an affected territory
12
. Management of rheumatoid vasculitis remains largely

emperical. Two open
-

label studies
13,14

as well as long
-
term clinical experience favour
the use of cyclophophomide and high
-
dose glucocorticoids in patients with seve
re
rheumatoid arthritis
. Overall, sustain treatment with

potent dise
a
se modifying ant
i
-
rheumatic drug is recommended
15.








Pat
ients and Methods

All patients that were seen in the rheumatology clinic were evaluated and diagnosed.
Twenty (20) cases of rheumatoid arthritis were seen over three and half years (July
2009
-
Dec. 2012). All patients that presented with synovitis that met
the American
College of Rheumatology criteria were included in the study. 14 females ( %) and 6

males

( %) were diagnosed as having rheumatoid arthritis. Laboratory investigations
requested were rheumatoid factor, anti
-

cyclic citrillunated peptide,
ESR, and full blood
count. Plain radiographs of the hands and feet were
also requested as necessary.

All the patients had elevated ESR. 10 ( %) patients had ESR level above 100mm/hr
and that corresponded with the degree of synovitis

in these patients. 8 ( %) patients
had significant rheumatoid factor titres, while 12( %) patients were nega
tive for
rheumatoid factor. Only

8 ( %) patients were able to process anti
-
cyclic citrillunated
peptide
antibodies and all the eight patient
s showed positive results. One of the patients
had extremely high titre value.

14 ( %) patients showed mild anaemia while 6 ( %)
had PCV above 30%.

6 ( %) patients had bony erosion of the digits on plain radiograph and one patient
showed erosion and

multiple amputations of the toes of both feet.














DISCUSSION

Rheumatoid vasculitis is a rare but

serious

complication of rheumatoid arthritis
16
.
Rheumatoid vasculitis

manifests almost exclusively in patients with rheumatoid auto
-
antibodies and often occurs in the context of other extra
-
articular manifestations. Auto
-
antibodies linked with rheumatoid vasculitis

include

rheumatoid factor and

anti
-
cyclic
citrullinated pep
tide (CCP)
17
.
Case control studies have suggested that in addition to
rheumatoid factor and CCP positivity, male gender, tobacco use, rheumatoid nodules

and older onset and long standing disease duration confer added risk
18
.

Rheumatoid vasculitis

may involve virtually any organ of the body
19
. The most common
site
s

of involvement are the skin and peripheral nerves. In many cases, the skin or
peripheral nerves are involved in more than 80% of patients

20
.

Cutaneous manifestations of rheumatoid vascu
litis include palpable purpura, nodules,
ulcers and digital necrosis
21
. When skin findings are present, a careful search for other
systemic manifestatio
ns is necessary to characterize

the s
e
verity of the vasculitis
presentation. Skin involvement without ot
her organ system involvement carries a more
favourable prognosis
22
.

The only patient with classical features of rheumatic vasculitis presented with past
history of recurrent nodules in the elbows and marked elevated ESR, and anti
-
CCP
antbodies
. Presence of rheumatoid nodules predict the occurrence of systemic
vasculitis

23,24
. The presence of anti
-
CCP antibodies in patients with rheumatoid arthritis
is associated with progressive joint damage

25
, as well as sever
e

extra
-
articular
manifestation
s
26
. The degree of joint damage
observed
in this patient will support the
earlier finding in such patients.


Recently, the diagnostic value of anti
-
CCP antibodies was evaluated in patients with
systemic vasculitis. In the mentioned study, anti
-
CCP antibod
ies were detected in 93%
of 25 patients with systemic rheumatoid vasculitis as against 70% in 19 patients with
rheumatoid arthritis but without vasculitis
27
.

Anti
-

CCP levels also tend to be higher in
patients with rheumatoid arthritis who have systemic
vasculitis than those who do not
26
.

The
ESR of

the

only

patient with vasculitic lesion was modestly elevated as compared
to active rheumatoid arthritis where the ESR is markedly elevated.
The simple
explanation is that this patient has developed burned
-
out


disease. Burned

out
-
disease simply means the arthritis is no more active.


Artherosclerotic vascular disease must be differentiated from rheumatic vasculitis.
Although Scott et al in 1984 proposed diagnostic criteria for systemic rheumatic
vasculitis

2
8
. B
iopsy of the lesion is still typically required for definitive diagnosis
12
.

The patient with vasculitic

lesion was first referred to the rheumatologist after 20 years
of onset of rheumatoid arthritis. Definitive treatment of rheumatoid arthritis must start
immediately when the diagnosis is made. It is known that, if definitive treatment is
delayed beyond 2
years of onset of the disease, bony erosion

and other poor prognostic
features would have developed and that portends poor prognosis.

Vasculitis is rheumatoid arthritis is generally associated with long standing disease, has
an important impact on patient’
s well
-
being, and markedly influences patient’s life
expectancy
29
.
The morbidity and mortality of rheumatoid vasculitis are substantial.
Studies have shown that the 5
-
year mortality rate is 30% to 50%, with even higher rates
of morbidity related to disease

complications or vasculi
tis treatment

related toxicity
30
.

Advances in the therapies for rheumatoid arthritis will likely continue to reduce the
incidence of vasculitis. Patients who develop leg ulcers and digital ulcers however,
should be more closely mon
itored, especially patients who have high titres of
rheumatoid factor and positive antibodies to cyclic citrillunated peptide (anti
-
CCP).

Conclusively, rheumatoid arthriti
s patients and patients with conne
ctive tissue disorders
should be promptly referred to the rheumatologist for an appropriate
assessment and
management.

















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