[Oxidative stress correction in the treatment of severe keratoconjunctivitis sicca in patients with Sjorgen's syndrome]. / Korrektsiia oksidativnogo stressa v lechenii tiazhelykh form sukhogo keratokon''iunktivita pri sindrome Shegrena.

PURPOSE: To evaluate the antioxidative effect of artificial tears in the treatment of keratoconjunctivitis sicca (KCS). MATERIAL AND METHODS: The study included 43 patients (60 eyes) with severe KCS: 38 women (50 eyes) and 5 men (10 eyes) aged from 27 to 76 years (in average 52 years). Patients were randomly divided into 2 groups; all patients used therapeutic silicone hydrogel soft contact lens (SCL) and instillations of 0.05% Cyclosporin A (CyA) 2 times a day. Patients of the first group (22 people, 30 eyes) used 0.15% hyaluronic acid. In the second group, patients (21 people, 30 eyes) used a tear substitute with similar surface-active component, but with antioxidant properties. The results of the treatment were evaluated using basic and additional research methods at 1, 3, 6 and 12 months. RESULTS: Corneal epithelialization was achieved after 1 month of conservative treatment in all patients who wore SCLs and were treated with instillations of 0.05% CyA and artificial tears. Then the lenses were removed and the patients were switched to instillations of CyA and artificial tears. Comparative analysis of the results showed that higher functional indicators were achieved in the second group, where patients used Artelac Rebalance drops with antioxidant properties. CONCLUSION: Improvement of clinical and functional parameters in the treatment of severe forms of KCS was achieved by direct action on both links of pathogenesis with minimal amounts of the drugs and the frequency of their use. Subsequent supportive therapy contributed to stabilization of the results and further increase of the functional parameters.

Noninvasive Electrical Stimulation for the Treatment of Chronic Ocular Pain and Photophobia.

INTRODUCTION: "Dry eye" or "keratoconjunctivitis sicca" is a multifactorial disease estimated to have a worldwide prevalence of 5-33%. Conventional therapies targeting the ocular surface with artificial tears, anti-inflammatories, punctal closure, eyelid hygiene, and antibiotics do not provide relief in all patients, especially those with neuropathic-like ocular complaints (wind hyperalgesia and photophobia). We anticipated that ocular transcutaneous electrical nerve stimulation (TENS) would alleviate symptoms of ocular pain, photophobia, and dryness in these latter individuals. METHODS: All individuals who received electrical stimulation between May 10, 2016 and April 6, 2017 for the treatment of chronic ocular pain at the oculofacial pain clinic of the Miami Veterans Administration Hospital were included in this retrospective review. All patients had symptoms of dryness along with other neuropathic-like symptoms (e.g., photophobia) and minimal signs of tear dysfunction. Ocular pain intensity, symptoms of dryness, and light sensitivity were compared pre-treatment and five min post-treatment via a two-tailed paired Student's t-test. RESULTS: The use of TENS significantly reduced the mean pain intensity in both the right and left eyes five min after treatment compared to prior to treatment (p < 0.05, paired t-test). The use of TENS significantly decreased light sensitivity in both eyes (p < 0.05). The findings for symptoms of dryness, however, were equivocal with a significant decrease in the left eye but not the right (p < 0.05, paired t-test). DISCUSSION: Our data indicate that TENS may similarly provide analgesia in patients with dry eye symptoms as it does for many other chronic pain conditions. Furthermore, the noted effect on symptoms of photophobia and dryness suggest that all may be linked by similar trigeminal-thalamic-cortical pathways. Prospective studies with electrical stimulation of dry eye are needed to further elucidate its benefit and mechanism of action.

Uveodermatologic syndrome concurrent with keratoconjunctivitis sicca in a miniature poodle dog.

A 5-year-old, intact female miniature poodle dog was presented with chronic ophthalmic problems, facial poliosis, nasal depigmentation and multiple areas of alopecia over the trunk. Bilateral uveitis preceded dermatologic signs by 5 months and gradually worsened. The dog was diagnosed as having uveodermatologic syndrome (UDS) concurrent with keratoconjunctivitis sicca (KCS). Treatment with immunosuppressive drugs resolved the dermatologic lesions, but uveitis continued to progress.

Syndrome uvéodermatologique concomitant à une kératoconjonctivite sèche chez un caniche miniature. Une chienne caniche miniature intacte âgée de 5 ans a été présentée avec des problèmes ophtalmiques chroniques, une poliose faciale, une dépigmentation nasale et plusieurs plaques d'alopécie sur le tronc. L'uvéite bilatérale a précédé les signes dermatologiques 5 mois auparavant et elle s'est graduellement aggravée. La chienne a été diagnostiquée comme atteinte du syndrome uvéodermatologique concomitant à une kératoconjonctivite sèche. Le traitement avec des immunodépresseurs a résolu les lésions dermatologiques, mais l'uvéite a continué de progresser.(Traduit par Isabelle Vallières).

Severe extra-glandular involvement and pleural effusions complicating primary Sjogren's syndrome: a case report.

BACKGROUND: Sjogren's syndrome, an autoimmune disease of the exocrine glands, results in keratoconjunctivitis sicca, xerostomia, and dental caries. It is often overlooked, considered by clinicians to be a benign disease. However, it can cause life-threatening extra-glandular complications that affect multiple organ systems. CASE PRESENTATION: Here we present a 78-year-old Caucasian woman with a history of primary Sjogren's syndrome (pSS) whose symptoms of keratoconjunctivitis sicca were managed managed conservatively. She was evaluated for sub-acute shortness of breath. Imaging showed severe bronchiectasis with features of lymphocytic interstitial pneumonia. She also had exudative bilateral pleural effusions and skin ulcers, likely vasculitic in origin. The workup was significant for anti-Ro antibody, pancytopenia, hypocomplementia, cryoglobulinemia and monoclonal gammopathy, all of which reflect disease severity. Although there was no evidence of malignancy, she developed B-cell non-Hodgkin lymphoma during follow-up. CONCLUSIONS: Primary Sjogren's syndrome can result in severe multi-organ disease. Pleural effusions are a rare complication of pSS, with only ten cases reported in the literature over the last 30 years, and tend to respond well to steroids. Prognostic biomarkers for disease severity include hypocomplementia, cryoglobulinemia, monoclonal gammopathy, and hypergammaglobulinemia. In this report we review the literature and the management of the disease.

Minor salivary gland transplantation for severe dry eye disease due to cicatrising conjunctivitis: multicentre long-term outcomes of a modified technique.

AIM: To report the clinical outcomes of autologous minor salivary gland transplantation (MSGT) for the treatment of severe dry eye disease caused by cicatrising conjunctivitis. METHODS: This was a retrospective case series of patients undergoing MSGT at four different centres from 2016 to 2018. The technical modifications included en bloc harvesting of a 20 mm×15 mm mucosa-gland-muscle complex and fixation of the glands to the superior bulbar surface anchored to the superior rectus muscle. The primary outcome measure was improvement in best-corrected visual acuity (BCVA). Secondary outcome measures were change in Schirmer test scores and grades of conjunctival and corneal fluorescein staining, grades of corneal neovascularisation, opacification and keratinisation. RESULTS: 21 eyes of 19 patients underwent MSGT, with a median follow-up duration of 3 years. The median BCVA improved from a baseline value of 20/500 to 20/125 at 1 year (p=0.0004) and 20/80 at 3 years (p=0.0002) after surgery. The proportion of cases with BCVA ≥20/200 improved from 38% at baseline to 67% at 1 year (p=0.0294), 78% at 2 years (p=0.0227) and 93% at 3 years (p=0.0015) after surgery. There was a significant improvement (p<0.0036) in Schirmer scores, conjunctival and corneal staining scores as well as grades of corneal neovascularisation and opacification after surgery. There were no serious sight-threatening complications in the transplanted eyes or at the donor site. CONCLUSIONS: Long-term improvement in the visual acuity, ocular surface environment, and keratopathy was noted after MSGT performed in severely dry eyes using a modified technique.

Sicca symptoms, and lacrimal and salivary flow in Albanian patients with rheumatoid arthritis.

BACKGROUND: Rheumatoid arthritis affects primarily the synovial joints, but is often accompanied by extra-articular manifestations, including lacrimal and salivary gland involvement. The aim of the present study was to estimate the prevalence of ocular and oral sicca symptoms and reduced lacrimal and salivary flow in rheumatoid arthritis and the relation between sicca symptoms and objective measures of lacrimal and salivary flow. METHODS: We examined 88 consecutive hospitalized patients with rheumatoid arthritis and 88 age- and gender-matched healthy controls. The examination included the standardized questionnaire for keratoconjunctivitis sicca and xerostomia which forms part of the European criteria for Sjögren's syndrome, the Schirmer's I test and measurement of unstimulated whole salivary flow rate. RESULTS: A significantly higher proportion of patients (40.9%) reported ocular sicca symptoms than healthy matched controls (8.0%). Further, a significantly higher proportion of patients (44.3%) reported oral sicca symptoms compared to controls (13.6%). 48.9% of the patients had low Schirmer I score, compared to 20.5% of controls. Reduced salivary flow was found in 27.3% of patients, compared to 9.1% of controls. The differences in lacrimal and salivary flow between patient and control group were statistically significant. The minimum prevalence of secondary Sjögren's syndrome was 14.8%. Weak association was observed between sicca symptoms and the objective measures of lacrimal and salivary flow in patients, and no association was detected in control subjects. CONCLUSIONS: Sicca symptoms and reduced lacrimal and salivary flow were common manifestations in rheumatoid arthritis and should be given adequate consideration during management of patients with rheumatoid arthritis.

Idiopathic sclerosing peritonitis.

Sclerosing encapsulating peritonitis (SEP) is a rare complication of chronic peritoneal dialysis and beta-blocker (practolol) usage. The authors report a case of idiopathic SEP developing in a 39-year-old woman with associated ovarian cysts and kerato-conjunctivitis sicca syndrome. These associations have not been reported previously. The histological diagnosis of SEP was made after laparotomy for intestinal obstruction. The patient continues to have chronic intestinal failure, managed conservatively by long-term parenteral nutrition and steroids for more than 12 years.

Optimising tear replacement rheology in canine keratoconjunctivitis sicca.

Dry eye is a substantial problem in a large number of human and canine patients. Numerous laboratory models for tear deficiency exist using genetically predisposed rodent models, animals treated with topical anti-muscarinics, or those kept in environments with increased air flow to produce the ocular surface changes seen in human patients. Canine keratoconjunctivitis sicca, seen in many thousands of dogs kept as companion animals, can provide a valuable spontaneous model for testing tear replacement medications that might better model disease in human patients, existing as it does in an outbred population that live in the same environments as their owners. Here the development of a crosslinked hyaluronic acid topical drop is described together with the results of trials on dogs with spontaneous keratoconjunctivitis sicca. Although hyaluronic acid in its native form in tear replacement drops shows a Newtonian rheology, the crosslinked product described here behaves in a non-Newtonian manner, with the same shear thinning shown by the tear film itself. The crosslinked product thus shows itself as a potentially valuable tear replacement medication for the human dry eye population as well as for dogs with the same condition.

The Use of Conjunctival Pedicle Flaps to Prevent Corneal Perforation in Graft-Versus-Host Disease.

PURPOSE: We report the clinical outcomes of two chronic graft-versus-host disease (cGVHD) corneal thinning cases that were successfully treated with conjunctival pedicle flaps in an effort to prevent impending corneal perforation. METHODS: The patients were treated with topical and systemic corticosteroids, therapeutic contact lenses, lubricants, and punctual plugs. Conjunctival pedicle flaps were used because conventional treatments had failed, and corneal perforation was imminent. RESULTS: Both patients were successfully treated with conjunctival pedicle flaps. Following conjunctival flap removal, one patient had corneal clarity restored following penetrating keratoplasty. CONCLUSIONS: Conjunctival pedicle flaps provided a strong barrier for the corneal surface and stabilized anterior chamber in cGVHD patients with impending corneal perforation following allogeneic hematopoietic stem cell transplant.

Bilateral recurrent calcareous degeneration of the cornea.

PURPOSE: To report clinical, histologic, and electron microscopic findings in several consecutive keratoplasties with recurrent pancorneal calcification in a patient with chronic graft versus host disease and severe keratoconjunctivitis sicca following bone marrow transplantation for chronic myelogenous leukemia. METHODS: Altogether 5 penetrating keratoplasties were performed in both eyes for descemetocele formation and corneal perforation as well as pancorneal calcification associated with severe visual loss. Histologic examination and electron microscopy were performed on the corneal buttons obtained. RESULTS: Histology and electron microscopy confirmed pancorneal extracellular calcium deposition with increasing severity in each consecutive keratoplasty. Calcification was associated with necrosis and inflammation of the corneal stroma. Clinically unsuspected fungal keratitis was observed in 1 specimen. CONCLUSIONS: Keratoconjunctivitis sicca, epithelial defects, corneal inflammation, and infection are most probably responsible for the recurrent pancorneal calcification seen in our patient. The role of cytokines in this complication is discussed.

Characterization of lacrimal gland lesions and possible pathogenic mechanisms of keratoconjunctivitis sicca in dogs with leishmaniosis.

In a previous study, it was found that 2.8% of dogs with leishmaniosis had keratoconjunctivitis sicca (KCS). The aim of this study was to characterize the lesions present in the lacrimal glands of dogs with leishmaniosis and to determine the presence of the parasite by means of immunohistochemistry. The inflammatory infiltrate was described as granulomatous or pyogranulomatous and was located around the ductal component of the glands. Immunoperoxidase staining localized the parasites following the same pattern. Samples from eyes that had clinical signs compatible with KCS presented inflammatory infiltrate and parasite more commonly than those from eyes without clinical signs. One of the mechanisms of KCS in dogs with leishmaniosis may be the inflammatory infiltrate located around the ducts of lacrimal glands, producing retrograde accumulation and retention of secretion. Meibomian gland was the most commonly affected by the infiltrate, highlighting the possibility of a qualitative KCS in these dogs.

Peripheral ulcerative keratitis in the setting of rheumatoid arthritis: treatment with immunosuppressive therapy.

Peripheral ulcerative keratitis (PUK) is a rare but serious inflammatory eye condition that can complicate rheumatoid arthritis. PUK can be a warning sign of impending vasculitis, and cytotoxic therapy may be necessary to induce remission. We have encountered three patients with PUK in the past year. Two patients had long-standing quiescent rheumatoid arthritis who developed photophobia. Diagnosis was made by slit lamp examination. Treatment with local cyclophosphamide and prednisone resulted in prompt remission of the ulcer within 8 weeks. Cytotoxic therapy was discontinued altogether within 6 months. The third patient was also treated successfully with oral steroids and azathioprine. In all patients, sicca was noted. None of them had any evidence of systemic vasculitis. PUK, when recognized early and treated aggressively, can result in remission of the ulcer and in the prevention of vasculitis. Keratoconjunctivitis sicca can accompany PUK independent of the activity of rheumatoid arthritis.

Conjunctival biopsy in scleroderma and primary Sjögren's syndrome.

Keratoconjunctivitis sicca complicates both scleroderma and Sjögren's syndrome. Scleroderma of recent onset is difficult to diagnose, but is easily recognized late in its course. To assess the value of the conjunctival biopsy in the diagnosis of scleroderma, we used light, scanning, and transmission electron microscopy to compare in a masked fashion specimens from 21 patients with scleroderma and 14 patients with primary Sjögren's syndrome. Epithelial changes permitted diagnosis of early scleroderma in the two groups of patients. Lymphocytic infiltrate was always present in Sjögren's syndrome, but not in scleroderma. Fibrosis was always found in scleroderma, even in scleroderma of recent onset, but was absent in Sjögren's syndrome. The fibrosis was distributed around capillaries in a band-like pattern and was associated with degranulating mast cells. Conjunctival biopsy is valuable for the early diagnosis of scleroderma and for differentiating between scleroderma and primary Sjögren's syndrome.

Slow-release artificial tear inserts in the treatment of dry eyes in patients with rheumatoid arthritis.

Twenty-two patients with rheumatoid arthritis and moderate to severe bilateral keratoconjunctivitis sicca were treated with artificial tears and a soluble slow-release artificial tear insert in a crossover trial. While using the inserts all but two patients reported an improvement in their symptoms. All the patients showed an improvement in the degree of conjunctival and corneal staining while using the inserts (p = 0.0001 and 0.00001 respectively). Nineteen patients (86%) found the inserts an acceptable form of therapy and 14 (64%) preferred them to other treatment regimens. Placement of the insert was not a problem despite all the patients having rheumatoid changes in their hands resulting in varying degrees of deformity. Two patients experienced initial difficulties with placement, but these were quickly resolved.

Autoimmune thyroiditis and primary Sjögren's syndrome: clinical and laboratory evidence of the coexistence of the two diseases.

The prevalence of primary Sjögren's syndrome (primary SS) among patients with autoimmune thyroiditis (AT), and that of AT among patients with primary SS were studied prospectively. Of 63 patients with AT, one had precipitating antibodies against SS-A/Ro antigen together with objectively verified primary SS, and none had precipitating anti-SS-B/La antibodies; in contrast 17/63 (27%) had above normal values of anti-SS-B/La antibodies, as tested with ELISA. Of 19 AT patients tested objectively for xerostomia and keratoconjunctivitis sicca, six (32%) had keratoconjunctivitis sicca together with xerostomia and four (21%) had autoimmune sialadenitis. The prevalence of AT in patients with primary SS (n = 28) was 18%; of the 28 patients, 64% had an enlarged or abnormal thyroid gland on palpation and four of them (14%) had cytology verified AT. Ten (36%) had anti-thyroglobulin and/or antimicrosomal autoantibodies. Based upon the present investigation we conclude that the prevalence of primary SS is ten times higher among patients with AT, and that of AT is nine times higher among patients with primary SS, compared with the general population.

Sjögren's syndrome in rheumatoid arthritis and progressive systemic sclerosis. A comparative study.

One hundred and eleven patients with rheumatoid arthritis (RA) and 44 with progressive systemic sclerosis (PSS) were prospectively evaluated for evidence of Sjögren's syndrome (Ss). The diagnosis was established when a patient with a lip biopsy focal lymphocytic infiltration score of greater than or equal to 2+ in Tarpley's scale had keratoconjunctivitis sicca (KCS) and/or xerostomia. Out of 44 RA and 10 PSS patients with positive lip biopsy, 34 and 9 had criteria for Ss respectively, suggesting a 31% prevalence of Ss in RA and a 20.5% in PSS. Six per cent of the RA patients spontaneously offered complaints of subjective xerophthalmia whereas 11.1% and 22.2% of those with PSS did so for subjective xerophthalmia and xerostomia respectively. However, specific questionnaire elicited subjective xerophthalmia in 38.2% and subjective xerostomia in 5.9% of the RA patients, whereas in 55.5% and 66.7% of the PSS ones respectively. Parotid gland enlargement was detected in 20.6% of the RA and in 44.4% of the PSS patients with Ss. Anti-Ro (SSA) antibodies were present in the sera of 23.5% and 33.3% of them respectively. Severe extraglandular manifestations were unusual in both groups. Our results suggest that, although both Ss in RA and that in PSS lack prominent exocrine gland symptomatology, certain differences between the two and similarities of the latter to primary Ss, would not justify the term 'secondary Ss' for the syndrome accompanying scleroderma, as it has been applied to that accompanying RA.

Keratoconjunctivitis sicca and corneal ulcers.

We reviewed the records of 56 patients (109 eyes) who satisfied our criteria for keratoconjunctivitis sicca (KCS) to determine factors associated with the development of sterile corneal ulcers. The patient's age, sex, and associated local ocular surface and/or systemic disease were studied. We found a statistically significant association between the development of corneal ulceration and the presence of an underlying condition or disease state, especially chronic rheumatoid arthritis. Patient sex and age were not significantly associated with the development of corneal ulceration. We present three case histories that illustrate the complications that arise in managing KCS plus an associated disease and methods of management.

Keratoconus-like topographic changes in keratoconjunctivitis sicca.

PURPOSE: To describe a case of inferior corneal steepening with a keratoconus-like pattern in a patient with nocturnal lagophthalmos and aqueous tear deficiency (ATD). METHODS: Axial curvature mapping was performed with the Tomey TMS-2N videokeratoscopy instrument. Keratoconus-like topography patterns were identified with the Klyce Software package of this instrument. RESULTS: Axial videokeratoscopy showed asymmetric inferior corneal steepening in the right eye. The surface regularity index (SRI), surface asymmetry index (SAI), and the simulated keratometric cylinder change (CYL) were 1.05, 5.05, and 0.75, respectively. The keratoconus screening indices were as follows for the right eye: Klyce/Maeda index of 95% similarity (clinical keratoconus interpreted) and Smolek/Klyce index of 23.09% severity (keratoconus suspect interpreted). Ultrasound pachymetric mapping showed a normal central corneal thickness. We found a similar topographic pattern of keratoconus in seven eyes of 74 dry eye patients who were previously evaluated with the Tomey TMS-2N. CONCLUSION: Chronic ocular desiccation and aqueous tear deficiency can produce inferior corneal steepening and high astigmatism resembling keratoconus.

Sterile corneal ulceration after cataract extraction in patients with collagen vascular disease.

We report the occurrence of sterile corneal ulceration in 11 eyes of eight patients with collagen vascular diseases and dry eyes after cataract extraction with intraocular lens implantation. Keratolysis occurred after both extracapsular and intracapsular cataract extraction and appeared unrelated to the type of intraocular lens. Despite aggressive lubrication and other medical treatment, including systemic immunosuppressive agents, penetrating keratoplasty was often required. Although all eyes were saved, visual outcome was usually poor. The histopathologic finding of polymorphonuclear leukocytes localized near the areas of corneal dissolution provides evidence for the role of polymorphonuclear leukocyte-derived collagenase as a contributing factor in the pathogenesis of sterile corneal ulceration in these patients.

Ocular flora of patients with AIDS compared with those of HIV-negative patients.

PURPOSE: To determine whether there are quantitative or qualitative differences in the ocular flora of patients with acquired immunodeficiency syndrome (AIDS) compared with human immunodeficiency virus (HIV)-negative patients. METHODS: Forty patients with AIDS and 42 HIV-negative controls were sex and age matched. All subjects had a detailed anterior segment examination, including Schirmer's test, rose bengal staining, and quantitative cultures of the conjunctiva and lids. Statistical evaluation of the relation between AIDS, keratoconjunctivitis sicca (KCS), and ocular flora was performed. RESULTS: No differences were observed in the types or numbers of organisms isolated from the conjunctiva or lids of patients with AIDS and HIV-negative subjects. Ocular flora was not influenced by use of systemic antibiotics, level of immunosuppression as measured by CD4 lymphocyte counts, KCS, or other ocular-surface disease. One AIDS patient was colonized by large numbers of Haemophilus influenzae OU with minimal clinical signs of inflammation or infection. CONCLUSION: There do not appear to be any differences in the ocular flora of HIV-negative patients and patients with AIDS. Presence of KCS and level of immunosuppression do not appear to affect the ocular flora in patients with AIDS.