Sclerodermatomyositis, ocular manifestations

Sclerodermatomyositis, ocular manifestations

a r c h s o c e s p o f t a l m o l . 2 0 1 7;9 2(7):334–337 ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA Short com...

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a r c h s o c e s p o f t a l m o l . 2 0 1 7;9 2(7):334–337


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Sclerodermatomyositis, ocular manifestations夽 M. Pedroza-Seres a,∗ , J.C. Serna-Ojeda a , L.F. Flores-Suárez b a b

Instituto de Oftalmología «Conde de Valenciana», Mexico City, Mexico Clínica de Vasculitis Sistémicas Primarias, Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico

a r t i c l e

i n f o

a b s t r a c t

Article history:

Background: Sclerodermatomyositis is an overlap syndrome of myositis and scleroderma,

Received 25 August 2016

with dermatological, muscular and joint involvement, but may also present with ocular

Accepted 5 October 2016


Available online 10 April 2017

Clinical case: A 57 year-old woman presented with ophthalmological manifestations, includ-


Oriented physical examination and laboratory studies led to the diagnosis, with the need


for systemic treatment.

ing scleral thinning 360◦ , and the presence of cells in the anterior and posterior chamber.

Overlap syndrome

Conclusion: Sclerodermatomyositis is a rare disease. Its diagnosis needs thorough clinical

Antinuclear antibodies

and laboratory studies, and its management should be multidisciplinary when inflamma-


tory ocular manifestations may be present.


˜ ˜ S.L.U. All rights © 2016 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana, reserved.

Esclerodermatomiositis con manifestaciones oculares predominantes r e s u m e n Palabras clave:

Antecedentes: La esclerodermatomiositis es un síndrome de superposición que tiene mani-


festaciones dermatológicas, musculares y articulares, y que puede presentar afección ocular.

Síndrome de superposición

˜ Caso clínico: Se presenta el caso de una mujer de 57 anos en quien la exploración oftal-

Anticuerpos antinucleares

mológica hizo evidente adelgazamiento escleral 360 grados, celularidad anterior y vítrea. La


exploración física orientada y los estudios de laboratorio permitieron sustentar el diagnós-


tico de esclerodermatomiositis, por lo que se trató de forma sistémica. Conclusión: La esclerodermatomiositis es una enfermedad rara, cuyo diagnóstico implica estudio clínico y de laboratorio, y su manejo debe ser multidisciplinario, donde las manifestaciones oculares inflamatorias pueden estar presentes. ˜ ˜ S.L.U. Todos © 2016 Sociedad Espanola de Oftalmolog´ıa. Publicado por Elsevier Espana, los derechos reservados.

夽 Please cite this article as: Pedroza-Seres M, Serna-Ojeda JC, Flores-Suárez LF. Esclerodermatomiositis con manifestaciones oculares predominantes. Arch Soc Esp Oftalmol. 2017;92:334–337. ∗ Corresponding author. E-mail address: [email protected] (M. Pedroza-Seres). ˜ ˜ S.L.U. All rights reserved. 2173-5794/© 2016 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana,

a r c h s o c e s p o f t a l m o l . 2 0 1 7;9 2(7):334–337



Clinic case report

Sclerodermatomyositis is an “overlap” syndrome, defined as having the concurrent presence of diseases that fulfill defined clinical data and diagnostic criteria independently, occasionally accompanied by specific serological tests.1–4 The etiopathogeny of dermatomyositis has been linked to the deposit of immune complexes in vessels and is regarded as a supplement-regulated vasculopathy.1,2 Clinical expressions include symmetric proximal weakness, myalgia, myositis, Gottron sign (erythematous or purple plates appearing in the dorsal area of the interphalanx, metacarpian-phalanx, elbows and knee joints) and calcinosis.1 Some ocular expressions have been described including heliotrope erythema (hyperpigmentation, scaling and palpebral edema), avascular areas in the conjunctiva, uveitis, scleritis and glaucoma, as well as retinopathy with cotton-like lesions, retinal hemorrhages, retinal edema and optic nerve paleness.5–8 Diagnostic is based on clinical findings, data compatible with inflammatory myopathy and antibody pattern. The auto-antibodies reported in this conditions include anti-ribonucleoprotein (anti-RNP) which are associated with this overlap in variable percentages (generally under 30%) whereas nearly 90% of patients with scleroderma are positive to antinuclear antibodies (AAN), and 35% are positive for rheumatoid factor.2,9,10 Treatment includes systemic glycocorticoids although generally an immunosuppressant agent is also required.

Female, 57, who consulted due to poor vision and intermittent episodes of throbbing pain in both eyes during 20 years. She was diagnosed in another institution with idiopathic panuveitis and treated with topical steroids and midriatics with apparent clinical improvement. Two years prior to consulting at the authors’ practice, the patient debuted with pain in proximal and distal inter-phalanx, metacarpo-phalanx and shoulder joints without morning stiffness and with proximal myalgia in thoracic limbs. In addition, she exhibited dyspnea with median effort and dysphagia against solids as well as lesions in the forehead which increased with exposure to the sun. Pathological personal history included hypertension with 7 years evolution. Ophthalmological examination produced visual acuity in the right eye (RE) of 20/200 that improved to 20/80 and 20/100 in the left eye (LE) that improved to 20/80. Biomicroscopy revealed 360◦ scleral thinning, cornea with discrete peripheral thinning, anterior chamber with cellularity 2+ and clear lens in the RE (Fig. 1). The LE showed sclera without alterations, anterior chamber with cellularity 2+, posterior synechiae and clear lens (Fig. 2). RE ocular fundus did not show alterations whereas the LE ocular fundus identified 2+ vitreous cellularity (Fig. 3). General physical examination found Gottron’s papules, heliotrope erythema, changes suggesting Raynaud in the distal phalanx of hands, increased skin consistency in those areas, Heberden and Bouchard nodules and abnormal capillaroscopy (Fig. 4).

Fig. 1 – Clinic images of the right eye, showing scleral thinning.


a r c h s o c e s p o f t a l m o l . 2 0 1 7;9 2(7):334–337

cutaneous induration, abnormal capillaroscopy and esophagic symptoms. In the most recent follow-up, the patient was in treatment with prednisone at a dose of 1 mg/kg/day, that was gradually diminished every week, azathioprine 100 mg daily, 1% prednisolone drops and reduction dose and midriatics every 12 h. The patient remained clinically stable with cellularity diminished to 0+ in anterior chamber and vitreous of authorities, and controls extraocular expressions in one year follow-up.

Discussion Fig. 2 – Clinic image of the left eye, showing posterior synechiae.

Fig. 3 – Clinic images of left eye fundus, showing turbidity due to vitreous cellularity.

The importance of the present case is due both to the infrequent appearance of overlap syndromes and to the scarcity of literature about ocular expressions thereof, in contrast with other self immune dermatological diseases. A study by Akikusa et al. reporting the ocular expressions of 108 patients with juvenile dermatomyositis found that 38% had heliotrope erythema, 18.3% had cataracts mainly attributed to the chronic use of glycocorticoids, 7.4% had palpebral atrophic scars and 4.9% had blepharitis.5 However, retinopathy associated to dermatomyositis is rare. It was first reported in 1958 by Venkatesh et al., with 2 sclerodermatomyositis cases with proliferative retinopathy. However, to the best of the authors’ knowledge, the case of the present patient is the first to be published with non-necrotizing anterior scleritis and non-granulomatous bilateral anterior uveitis with posterior expressions.6 The long history of ocular symptoms diagnosed 20 years before must be noted. This paper has described some inflammatory ocular expressions which are not typical in a sclerodermatomyositis case and that have contributed to the differential diagnostic. As said disease is rare, its identification requires significant diagnostic suspicion as well as an extensive clinic and laboratory approach that includes full ophthalmological examinations. The prognostic role of ocular findings is yet to be defined.

Conflict of interests No conflict of interests was declared by the authors.


Fig. 4 – Gottron’s papules.

The relevant lab results were: anti-centromere antibodies 1:1.280 (normal: 1:20), AAN positive (59.7 U/ml, with normal <10 U/ml), PCR 20.7 mg/l (normal: <3.8), VSG 38 mm/h, CPK 310.7 U/l (normal: up to 140), rheumatoid factor, antibodies against Ro and La negative and normal blood biometry. Considering the clinic and serological findings, the patient was diagnosed with probable sclerodermatomyositis, based on typical dermatological changes, myalgia, high CPK, distal

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