Sarcoidosis is a multisystem disorder of unknown cause, characterized by noncaseating granulomatous inflammation.1 It affects the parenchyma of almost any organ, and thoracic symptoms are those most frequently observed. Overall, 30%–60% of the patients have eye involvement. It is usually bilateral uveitis, although, uveitis can occasionally develop in the absence of systemic disease, or could be the predominant manifestation with no significant extraocular impact.2–4 In clinical practice, the diagnosis of systemic sarcoidosis is based on the combination of clinical, radiographic and histopathological findings. The diagnostic criteria proposed by the First International Workshop on Ocular Sarcoidosis (FIWOS) were recently validated.5 These criteria classify ocular sarcoidosis as definite (uveitis and positive biopsy), presumed (bilateral hilar lymphadenopathy and uveitis without biopsy), probable (3 ocular signs and 2 laboratory findings without biopsy or bilateral hilar lymphadenopathy) and possible (4 ocular signs and 2 laboratory findings with a negative biopsy), in accordance with specific ophthalmological and laboratory findings.6 The latter are especially useful when there is no biopsy or the biopsy is negative.

The purpose was to categorize those patients with a diagnosis of ocular sarcoidosis from 2009 to 2014 in Hospital Universitario Donostia, in the province of Guipúzcoa in northern Spain, using the diagnostic and validated criteria of the FIWOS.

We retrospectively reviewed the case reports of the patients diagnosed with ocular sarcoidosis. The diagnosis of sarcoidosis was based on a combination of the clinical, radiographic and histological criteria of the FIWOS, as well as the exclusion of other granulomatous diseases like tuberculosis.

The variables recorded were patient age and sex, presence of systemic manifestations, anatomical location of the inflammation, tuberculosis screening, positive test for angiotensin-converting enzyme, lymph node biopsy, chest radiographic findings and treatment. To classify uveitis we used the anatomical classification of the Standardization of Uveitis Nomenclature (SUN) Working Group7 (Table 1). We excluded patients who had a positive result on the Quantiferon® test. Using these findings, we categorized the patients according to the FIWOS.

We found a total of 11 patients with a diagnosis of sarcoid uveitis, 7 women (63.6%) and 4 men (36.3%). The median age±standard deviation was 58±20.5 years. In 7 patients (63.6%), the eye was the first organ to be affected: 5 of them later developed the systemic clinical signs and 2 had only ocular clinical features. Four patients (36.3%) began with extraocular systemic manifestations, which, in order of frequency, were: bilateral hilar lymphadenopathy in 81.8%, respiratory manifestations in 36.3%, peripheral lymphadenopathy in 18.1%, parotitis in 27.2% and erythema nodosum in 9%. The locations of inflammation most frequently observed were chronic bilateral panuveitis in 54.5%, unilateral chronic anterior uveitis in 27.2% and bilateral chronic anterior uveitis in 18.1%. When the patients were categorized, 4 (36.3%) had a definite diagnosis of sarcoidosis, 5 patients (45.4%) had a presumed diagnosis, 1 patient (9%) had a probable diagnosis and 1 patient (9%) had a possible diagnosis of sarcoidosis.

Table 2 shows the above-mentioned variables in the patients diagnosed with ocular sarcoidosis.

The diagnosis of systemic sarcoidosis is presently based on a combination of clinical and radiographic findings, together with the histological confirmation and the exclusion of other granulomatous diseases, such as tuberculosis.8 In clinical practice, the determination of the angiotensin-converting enzyme level, serum calcium, plain chest X-ray, scintigraphy and screening tests, like Mantoux or Quantiferon®, initially indicate involvement of systemic sarcoid. Ocular involvement in sarcoidosis is presented by up to half of the patients and may even be the initial manifestation of the disease. Sarcoidosis can affect the lacrimal glands and cause keratoconjunctivitis sicca, involving the iris, as well, causing different forms of uveitis and even the development of optic neuritis when it affects the posterior pole. The anatomical location of inflammation in sarcoid uveitis mainly, and chronically, involves the posterior segment. It frequently consists of recurrent bilateral uveitis, and can even cause generalized involvement in the form of bilateral panuveitis associated with retinal vasculitis.9,10 The findings most frequently observed in uveitis associated with sarcoidosis include mutton fat keratic precipitates, iris nodules, tent-shaped peripheral anterior synechiae, vitreous opacities like “snowballs”, multiple peripheral chorioretinal lesions and nodular periphlebitis (Fig. 1). Moreover, there were other ocular signs that could indicate sarcoid involvement, especially if evaluated in the presence of ancillary tests that are positive for sarcoidosis11–14 (Table 3). The diagnostic criteria proposed by the FIWOS were validated in 2010. They include several ophthalmological signs indicative of sarcoidosis and comprise ancillary tests5 (Table 4). These criteria do not include invasive procedures and are useful, in clinical practice, when there is no confirmatory biopsy or when the biopsy is negative.

Fig. 1.

(A) Sequelae of anterior uveitis with tent-shaped peripheral anterior synechiae. (B) Multiple chorioretinal lesions in peripheral retina.

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Ocular and systemic manifestations of sarcoidosis, as well as the ancillary tests, show a great variability, a fact that complicates the diagnosis. With our work, we have put into practice the FIWOS criteria, which enabled us to categorize the patients with systemic clinical signs indicative of sarcoidosis together with other ocular manifestations and positive ancillary tests. Because of the heterogeneity of this disease, not all the patients fulfilled the diagnostic criteria for sarcoidosis; for this reason, after the diagnostic categorization, we see that, after satisfying the clinical and ophthalmological criteria and that of the ancillary tests, the patients’ disorder was classified as sarcoidosis. Most of our patients, coinciding with the literature, were women (63.6%). Ocular problems were the initial symptom in 7 of the overall group and most of them had ocular manifestations indicative of sarcoid disease. Tuberculin tests were negative in all of the patients and only 3 patients had a compatible lymph node biopsy. Those who had not undergone a biopsy for the classification criteria, after categorization, had a “presumed” diagnosis of sarcoidosis. The ophthalmological data showed that the most common anatomical location was chronic bilateral panuveitis, affecting 54.5% of the patients. In second place, unilateral chronic anterior uveitis, with 27.2%, was the most frequent form. Most of the patients began with clinical manifestations of uveitis, developing the systemic clinical disease later on. After the categorization of the patients, the diagnosis of sarcoidosis was definite in 4 patients, presumed in 5, probable in 1 and possible in 1 other patient.

The present study is especially interesting for rheumatologists who work in collaboration with ophthalmologists in uveitis clinics, as sarcoidosis is an uncommon disease, it is best to standardize criteria for the correct diagnosis of this disorder. We should also point out that the use of these criteria enables the diagnosis without the need for a biopsy, which could be iatrogenic or difficult in certain patients.

These criteria categorize patients with data indicative of sarcoidosis and who, because of the variability in the clinical course and organic involvement, have not been categorized as patients with sarcoid disease. The sarcoid ocular involvement plays a very significant role for categorization and, for this reason, it is important to stress a detailed ophthalmological examination in search of ocular signs indicative of a granulomatous inflammatory disease.

None.

The authors declare they have no conflicts of interest.