Systemic sclerosis is a connective tissue pathology with very heterogeneous clinical manifestations, associated in a small percentage with inflammatory eye diseases. In the specific case of uveitis, only isolated cases have been reported in the literature, especially in relation to the CREST syndrome. We present the case of a 53-year-old woman with CREST syndrome and chronic anterior uveitis, which we consider of clinical relevance given its low prevalence.
La esclerosis sistémica es una conectivopatía con unas manifestaciones clínicas muy heterogéneas, relacionándose en un pequeño porcentaje con enfermedades oculares inflamatorias. En el caso concreto de las uveítis, únicamente se han descrito casos aislados en la literatura, sobre todo con relación al síndrome de CREST. Presentamos el caso de una mujer de 53 años, con síndrome de CREST y uveítis anterior crónica, que consideramos de relevancia clínica dada su baja prevalencia.
Systemic sclerosis (SSc) is a connective tissue disease characterised by 3 pathological processes: fibrosis (essentially cutaneous and pulmonary), small vessel vasculopathy and specific autoantibodies.1 It can be classified as diffuse and limited (lcSSc) depending on the extent and distribution of the skin manifestations. LcSSc is frequently associated with CREST syndrome (Calcinosis cutis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasia).2 In this syndrome, ocular involvement is infrequent, the case presented below is the fourth described in the literature on uveitis with CREST syndrome.3–5
Description of the caseA 53-year-old female, housewife, no family history of interest, no known drug allergies or toxic habits, with a history of Hashimoto's thryoiditis and multinodular goiter. Followed up by rheumatology for CREST syndrome and secondary Sjögren's syndrome, under treatment with nifedipine, esomeprazole, cinitapride and levothyroxine.
Complementary studies showed titre positive ANA (1/2560) centromere pattern and capillaroscopy with sclerodermal pattern. No anomalies were observed on chest X-rays, functional, respiratory and diffusion tests or transthoracic echocardiograph.
The patient attended ophthalmology consultations for repeated episodes of red eye, pain and loss of right eye vision. On examination, she presented a non-hyperaemic eye with some isolated cells inside the anterior chamber (Tyndall 1+ and negative flare),6 synechias and pigment deposits in anterior crystalloids, together with cystoid macular oedema with visual acuity (VA) .5 in the right eye (RE); and was diagnosed with chronic anterior uveitis with secondary macular oedema. She was prescribed cycloplegic and topical corticosteroids (dexamethasone eye drops and subtenon's injection of triamcinolone acetonide) with progressive improvement. The patient continued to experience new flare-ups, coinciding with decrease and/or withdrawal of the corticosteroid; and therefore in approximately one year, oral methotrexate was started (15mg/week). After 5 months it was necessary to increase the dose (20mg/week/sc) and then discontinue it due to elevated transaminases after 2 months of treatment. She has remained stable since that time, 5 further flare-ups presented after that, and she started oral cyclosporine (50mg/12h) with improvement and clinical stabilisation until now (VA .7 in RE and .8 in LE).
Discussion of the caseUveitis consists of inflammation of the uvea, the middle portion of the eye, comprising the iris and the ciliary body (anterior part) and choroid (posterior part); although, in general terms, it includes any eye inflammation. Its principal aetiologies are: infections, immune-mediated systemic diseases (40%), syndromes confined to the eye and idiopathic (up to 30%). Most notable among the immune-mediated aetiologies are spondyloarthritis, sarcoidosis, Behçet's syndrome and inflammatory bowel disease, among others.
As for the ocular manifestations of SSc, the most notable are the palpebral alterations due to fibrotic changes, which occur more frequently in young patients, with greater skin involvement and earlier onset of the disease. Other ocular manifestations frequently related to this disease are: pinguecula, keratoconjunctivitis sicca, cataracts and blepharitis. Inflammatory ocular manifestations have been described, although much less frequently: of interest is the recent description of 2 cases of CREST syndrome with retinal vasculitis.7 We should also mention that in the study by Gomes et al.8 only 4.4% (2 patients) episcleritis was found and no cases of uveitis were described. In fact, this combination (uveitis and SSc) is very rare and only isolated cases have been described in the literature: 3 in patients with CREST syndrome,3–5 one in a patient with SSc9 and another in a patient with SSc without sclerodermia.10 although the frequency of this association is low, we believe that it should be taken into consideration when managing these situations in order to deliver multidisciplinary treatment in which ophthalmologists would play an important role.
Conflict of interestThe authors have no conflict of interest to declare.
Please cite this article as: Fuente Cosío S, Arca Barca B, Martínez García P, Sampedro López A. Uveítis anterior crónica en paciente con síndrome de CREST. Reumatol Clin. 2020;16:497–498.
