Scleritis and aortic incompetence. Two manifestations of connective tissue disease.

Abstract

From the Departments of Rheumatism Research and Cardiology, Manchester Royal Infirmary, and the Manchester Royal Eye HospitalScleritis is known to occur as a manifestation of connective tissue disease, particularly rheumatoid arthritis and polyarteritis nodosa (Manschot, 1961).In a recent study of 31 patients with scleritis (Lyne and Pitkeathly, 1968), evidence of connective tissue disease was found in fourteen (45 per cent.). Since this study was completed, we have encountered a further three patients presenting with scleritis in whom aortic incompetence was found. Two had severe aortic regurgitation and complete atrioventricular (AV) block. The third had a diffuse arteritis, and the aortic incompetence was only a minor feature of this illness.Case reports Case 1, a 56-year-old housewife, gave a 5-year history of almost continuous inflammation affecting the left eye with severe pain at times. The right eye had never been involved. For 2 years she had complained of breathlessness, weakness, tiredness, and light-headedness on exertion. During the previous few months, she had experienced episodes of dizziness and had lost consciousness on several occasions. Examination The abnormalities were confined to the left eye and the cardiovascular system.The perilimbal sclera of the left eye showed diffuse thinning for about 2 mm. all round. The sclera elsewhere was thickened by a subconjunctival granulomatous-like infiltration. A fairly severe uveitis was present. There was elevation of the retina on the temporal side subjacent to the site of the most intense inflammation.The pulse was regular, collapsing in type, and the rate 38/min. The blood pressure was 220/40 mm. Hg. A hyperdynamic left ventricular impulse was palpable. There was a short aortic ejection systolic murmur and a moderately long aortic diastolic murmur. The aortic second sound was diminished.

Scleritis and rheumatoid arthritis.

“…Exacerbations of the scleritis often coincided with the appearance and exacerbation of the systemic complications. In contrast with the patients described by Pitkeathly, Howitt, and Lyne (1970) none of our series had aortic incompetence.…”

Episcleritis and scleritis. A study of their clinical manifestations and association with rheumatoid arthritis.

“…It is possible that the changes, which include bundle-branch block myocardial infarction, and non-specific S-T wave changes, might have been in part, at least, due to rheumatoid involvement of the heart, but without necropsy it is impossible to differentiate between rheumatoid disease, induced ischaemia, and other causes of ischaemic heart disease (Hart,I969). Pitkeathly, Howitt, and Lyne (1970) documented three cases of aortic incompetence in patients with scleritis, one of whom had a history of polyarthritis of uncertain origin. They presented the scleritis and aortic incompetence as two manifestations of connective-tissue disease.…”

Not So Classic: An Unusual Presentation of a Rare Disease

“…Long et al highlighted a 16% incidence of giant cell arteritis (GCA) in scleritis and a couple of case reports highlight scleral inflammation as one of the initial symptoms in patients with Takayasu arteritis . Other diseases that can present with scleral inflammation and large vessel vasculitis are syphilis, tuberculosis, ankylosing spondylitis, Behçet's disease, rheumatoid arthritis (RA), granulomatosis with polyangiitis (Wegener's) (GPA), relapsing polychondritis, and Cogan's syndrome . Based on our patient's initial presentation of aortitis and scleritis, we included syphilis, tuberculosis, RA, GCA, GPA, Cogan's syndrome, and sarcoidosis in our differential diagnosis list.…”