Abstract:
Background: Rheumatoid arthritis(RA) is a systemic autoimmune disease
characterized by inflammation of the synovial membrane of joints.
However, RA can be associated with extra-articular manifestations
including vasculitis that occurs exceptionally in the retina.
Objectives: We aimed to show two retinal vasculitis(RV) cases as a
direct consequence in active RA patients.
Results: Case1:A 45-year-old man, followed for an immunopositive and
erosive RA, developed bilateral occlusive venous RV. It occurred
concomitantly with a flare-up of his RA and was treated with high doses
of prednisolone. Recently, the patient had a recurrence of RV, and his
RA was reactivated. Etiological workup of RV, including infectious
markers, antinuclear antibodies and autoantibodies to ANCA, was
negative. The rheumatoid origin was retained in the presence of severe
disease activity with strongly positive rheumatoid-factor(RF). The
patient was started on intravenous corticosteroid therapy and was
transitioned oral corticosteroid with favorable evolution. Since the
visual prognosis is involved an immunomodulatory therapy treatment was
considered.
Case2:A 33-year-old woman with a history of bilateral retinal detachment
treated surgically, followed for immunonegative and erosive RA,
presented with left RV complicated by a preretinal hemorrhage.
Etiological workup, including anti-nuclear antibodies and other common
infectious and autoimmune markers, was negative. An MRI of the hands was
performed showing bilateral active synovitis. Rheumatoid origin of RV
was retained. Systemic steroid therapy was initiated with articular and
visual improvement.
Conclusion: The retina should be examined for evidence of vasculitis in
rheumatoid disease and RV should be kept in mind as an ocular
complication of RA.
Introduction: Rheumatoid arthritis (RA) is a chronic systemic
autoimmune disease that is characterized by significant inflammation of
the synovial membrane of joints. However, RA can be associated with many
extra-articular manifestations including vasculitis that occurs
exceptionally in the retina.
We report here two cases of retinal vasculitis as a direct consequence
of rheumatoid disease in patients with active RA.
Case1: A 44-year-old man has been followed since 2012 for an
immunopositive and erosive RA treated by methotrexate at a dosage of
15mg/week and on oral corticosteroid therapy at a dosage of 10 mg/day of
prednisone equivalent with a good initial response. Since 2018 the
patient has stopped his treatment on his own initiative. In March 2021,
he presented with a sudden bilateral blurred vision and myiodesopsias of
the right eye. These symptoms were induced by bilateral venous occlusive
retinal vasculitis and intravitreal haemorrhage of the right eye. The
patient was given a pulse of solumedrol 1g/day for three consecutive
days without incident and was putted back on his background treatment of
methotrexate 15mg/week. The evolution was marked by a clear improvement
of his retinal vasculitis especially since the patient benefited from a
laser photocoagulation with his ophthalmologist. Currently, since August
16, 2021, the patient has presented a recurrence of his retinal
vasculitis with a right vitreous hemorrhage, hence his
re-hospitalization for solumedrol pulses. This ocular involvement was
concomitant with a relapse of his RA, which was due to poor compliance
with the treatment. The patient had no other extra-articular
manifestations (pulmonary, cutaneous, digestive, and neurological).
The blood count showed a hyperleukocytosis of 13300/µL (PNN 11270,
lymphocytes 1360/µL) with a hemoglobin of 13.3 g/dL and platelets of
254000/µL. C-reactive protein (CRP) was 10mg/L. Erythrocyte
Sedimentation Rate (ESR) was mildly elevated (29 mm).
As part of the etiological work-up for bilateral venous occlusive
vasculitis, a comprehensive evaluation was initiated. Firstly, we agreed
to eliminate an infectious origin by carrying out serologies (EBV, CMV,
toxoplasmosis, syphilis, Lyme disease and cat scratch disease) as well
as the search for Koch’s Bacillus in the sputum and QuantiFERON for
tuberculosis. The results of serologies for EBV and toxoplasmosis have
shown a long-standing immunity. Other infectious markers were all
negative. Secondly, a systemic origin (sarcoidosis, Behçet disease, ANCA
vasculitis, connectivitis) was suggested. A conversion enzyme assay was
unremarkable at 7IU/L. Antinuclear antibodies and autoantibodies to
SS-A, SS-B, pANCA and cANCA were all negative. Therefore, rheumatoid
origin of retinal vasculitis was retained in the presence of a flare-up
of rheumatoid disease (DAS28-ESR=4.4) with strongly positive rheumatoid
factor (RF) (200 UI/ml) and anti-citrullinated protein antibody (ACPA)
(181,51 RU/mL).
Accordingly, the patient was started on pulses of intravenous Solumedrol
at a dose of 1g/day for 3 days. The evolution was marked by a clear
improvement of the visual blur and persistence of the myiodesopsias. A
relay with oral corticosteroid therapy at 1mg/kg per day of prednisone
equivalent was started with methotrexate at a dosage of 20mg/week.
case 2 : a 33-year-old woman with a history of bilateral retinal
detachment treated surgically in 2014, has been followed since 2016 for
immunonegative and non erosive RA treated with methotrexate up to 20
mg/week with good progression. In July 2020, the patient had noticed
blurred vision with floating spots in the left eye. Ophtalmologic
examination revealed left retinal vasculitis complicated by a preretinal
hemorrhage. Simultaneously, the patient had progressive joint stiffness
of the wrists and hands for two weeks; these symptoms were most
prominent in the morning and seemed to improve as the day progressed. At
examination, she manifested tender and swelling of metacarpophalangeal
joints.
The blood count showed a hemoglobin of 11,3 g/dL and platelets of
304000/µL. CRP was 20 mg/L. ESR was mildly elevated (22 mm).
The diagnosis of RA was challenged, since there was nor erosion neither
immunological markers.
An exhaustive etiological workup was undertaken. Infectious origin was
eliminated by carrying out serologies (EBV, CMV, toxoplasmosis,
syphilis, Lyme disease and cat scratch disease) as well as the search
for Koch’s Bacillus in the sputum and QuantiFERON for tuberculosis. The
results were all unremarkable. Antinuclear antibody and antineutrophil
cytoplasmic antibody, lupus anticoagulant, and anticardiolipin antibody
were all negative. Rheumatoid factor (RF) and anti-citrullinated protein
antibody (ACPA) were also negative.
A magnetic resonance imaging (MRI) of the hands and wrists was performed
showing bony erosions at the metacarpophalangeal joints with bilateral
active synovitis which were typical for RA. Thus, the rheumatoid origin
of the retinal vasculitis was retained in the presence of active
rheumatoid disease concomitantly to ocular symptoms.
The patient was put on pulses of intravenous methylprednisolone sodium
succinate therapy for 3 consecutive days then relayed with oral
corticosteroid at 1mg/kg per day of prednisone equivalent with a marked
clinical improvement and disappearance of joint signs and attenuation of
eye signs.