1/5. Rosai-Dorfman disease manifesting as relapsing uveitis and subconjunctival masses.Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy, SHML) is a rare, non-hereditary, benign histiocytic proliferative disorder mainly affecting young people with common clinical characteristics such as painless bilateral cervical lymphadenopathy, fever, leukocytosis and polyclonal gammopathy. Extranodal manifestations have been reported in 28-43% of cases. Eye involvement is relatively uncommon (8.5%), and most of cases have presented as lymphoproliferation in the soft tissues of the orbit and eyelids. uveitis is an even more rare presentation as a review of all the literature. We describe a 63-year-old man with SHML with unusual ophthalmic manifestations of relapsing uveitis and bilateral subconjunctival masses. The results of biopsies were compatible with the characteristic histopathological findings of SHML: focal aggregations of S100-positive foamy histiocytes and the existence of lymphocytophagocytosis. During the clinical course, the patient relapsed but the relapse was relatively benign and the patient showed fair response to topical as well as systemic corticosteroid treatment. Here we describe this unusual presentation of SHML to inform physicians of the possibility for this systemic granulomatous disease to contribute to relapsing uveitis.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
2/5. sarcoidosis or Sjogren syndrome? Clues to defining mimicry or coexistence in 59 cases.We present 5 new cases of coexisting sarcoidosis and Sjogren syndrome (SS) and review the literature for additional cases in order to analyze the clinical, immunologic, and histologic characteristics that may help physicians differentiate the mimicry of SS by sarcoidosis from a true coexistence of both autoimmune diseases. We considered the coexistence of sarcoidosis with SS to be when patients presented specific histologic patterns of both diseases, simultaneously or at different times.Fifty-nine patients were included in the analysis (54 identified in the literature search plus our 5 unpublished cases): 49 (83%) patients were female and 10 (17%) were male, with a mean age at diagnosis of 50 years. According to the histopathologic examination of the exocrine glands performed in 53 cases, we defined coexistence of sarcoidosis and SS in 28 cases, while in the remaining 25 patients, sarcoidosis mimicked SS. Clues to identifying when sarcoidosis coexists with SS were a higher prevalence of systemic manifestations (arthritis and uveitis) and positive immunologic parameters (antinuclear antibodies, rheumatoid factor, and anti-Ro/SS-A), as well as the existence of a focal sialadenitis (Chisholm-Mason score grades III-IV, with a CD4 lymphocytic infiltration) in the salivary gland biopsy. In patients first diagnosed with primary SS, the appearance of some clinical features such as hilar adenopathies, uveitis, or hypercalcemia leads to the diagnosis of coexisting sarcoidosis. A careful application of the new American-European consensus criteria had a sensitivity of 93% and a specificity of 92% in identifying when SS coexists with sarcoidosis.In conclusion, the association of sarcoidosis with SS leads to a true coexistence of both diseases in more than half the patients described in the literature, while in the remaining patients, sarcoidosis mimics SS. In light of these results, sarcoidosis should not be considered as an exclusion criterion for the diagnosis of SS, and in patients with a suspected overlap of the two diseases, application of the new American-European consensus criteria for diagnosis of SS should be mandatory.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
3/5. Endogenous uveitis: current concepts of treatment.The medical treatment of patients with uveitis often necessitates the use of systemically administered drugs, including corticosteroids, cytotoxic agents, and cyclosporine. Because of the potential side effects of these medications, physicians unfamiliar with inflammatory ocular disease may be asked to participate in the care of these patients. The most important criteria on which to base the decisions of when and how to treat patients with uveitis are the site and severity of the inflammation, the degree of visual acuity, and the potential for restoration of vision with treatment. Slit-lamp examination, indirect ophthalmoscopy, and fluorescein angiography are useful for evaluating the loss of vision in patients with endogenous uveitis. The main goal of treatment is to prevent permanent scarring and deterioration of vision as a result of the disease or the drug side effects. Frequent reassessment will help ensure adequate treatment and will minimize adverse drug effects.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
4/5. psoriasis and intraocular inflammation.Presented in this series were seven men and three women. Ages when seen, ranged from 32 to 68 years (average 54). psoriasis had begun in childhood in the women and in the late 20's and 30's in the men. arthritis (ankylosing spondylitis) was present in only one. Their ocular inflammations began from ages 26 to 62 (average 41). The onset of the inflammation was acute iritis in four and in indolent iridocylcitis in six. All but one were bilateral and chronic. The vitreous had heavy debris in nine of the ten patients. The retina was normal in only three. Boggy congestion was present in two with cystoid edema. Patches of edema. fluorescein leaking, depigmentation of both maculae, pars plana exudate, and retinal vessel obliteration to grey-white, shaggy cords was present in at least one of the remaining five patients. Systemic corticosteroid therapy has been used in eight of the ten patients described in this report. Doses no higher than 30 mg of prednisone per day were used to initiate reversal of the inflammatory response. In case 10, knowledge of the sensitivity of the process to steroids led to the successful rapid reversal of a recalcitrant iritis with only 20 mg of prednisone as a first dose and 20 mg per day for less than three weeks. maintenance corticosteroid therapy ranged from 40 mg of prednisone every other day in case 9, prednisone 12.5 mg one day and 5 mg the next in case 8, to 8 mg of Aristocort or methylprednisolone acetate (M-edrol) daily in cases 1 and 2. In summary, these patients are older, have an indolent onset bilateral uveitis with dense vitreous debris, retinal abnormalites, and are extremely sensitive to systemic corticosteroids. Many of these patients had undergone the series of clinical evaluations known as a "uveitis survey." Many different systemic abnormalities were found and merited treatment which rarely made a difference in their ocular disease, though two improved after infected teeth were treated. Assuming that these ocular diseases were related to psoriasis, one can then challenge the value of anything found by a "uveitis survey." The author uses information gained from such studies to insure that the patient is in the best possible heatlh and then makes clinical correlations such as presented in this paper. The rarity of this combination of ocular inflammations in patients with psoriasis makes it risky to propose that this is a significant association. Arguments that this is a disease entity began with the clinical similarities: older age, indolent onset, vitreous and retinal involvement, and the extreme sensitivity to systemic corticosteroids. None of these patients had psoriatic arthritis and only one had ankylosing spondylitis. The best explanation for the fact that this disorder has not been emphasized in the past would be an attitude of ophthalmologists and physicians that the eye and skin disease were coincident.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
5/5. iris retraction syndrome after intraocular surgery.BACKGROUND: The authors report the postoperative development of iris retraction syndrome in two individuals. This is a rare syndrome that can present with pupillary block after an unrepaired rhegmatogenous retinal detachment. patients: The authors report two patients who had the iris retraction syndrome shortly after intraocular surgery. One patient had pupillary block that developed 6 months after cataract extraction and posterior chamber intraocular lens implantation. A second patient had uveitis, hypotony, and pupillary seclusion 4 weeks after pars plana vitrectomy. RESULTS: Both patients were treated successfully with a scleral buckling procedure and peripheral iridectomy. CONCLUSION: Postoperative uveitis that occurs with iris retraction and pupillary seclusion should alert the physician of an occult retinal detachment and warrant a thorough dilated funduscopic examination. Features unique to this report include the development of iris retraction syndrome in the presence of a pseudophakos, the rapidity of onset of this disorder after retinal detachment, and its masquerade as a persistent postoperative uveitis.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |