Knee joint synovitis in Behçet's disease: a sonographic study.

OBJECTIVE: The aim of this study was to investigate, using ultrasound (US), knee involvement in patients with Behçet's disease (BD). METHODS: Knee US was performed in 30 unselected Italian BD patients. Signs of arthritis (joint effusion, synovial proliferation) and presence of bone erosions and Baker's cysts were recorded. Power Doppler evaluation was performed. A semi-quantitative score was used for each structure examined, whereby 0 was to indicate the absence of any change and score from 1 to 3 the presence of mild to severe changes. A sum of the single scores was obtained. Disease activity was evaluated to identify the correlation with the US modifications. RESULTS: Twenty (66.6%) patients had symptomatic articular involvement and US showed knee involvement in 18 of them (60%). Synovial proliferation was detected in 14 (46%, positive power Doppler in 4), joint effusion in 14 (46%), bone surface erosions in 3 (10%). Four patients, asymptomatic for joint involvement, showed US alterations. Eleven patients showed a total score between 1 and 3, while the other 7 had a score between 4 and 6. Subjects with a higher US score presented an increased prevalence of acneiform skin lesions with respect to the group with US score 1-3. Statistical analysis showed a positive correlation between disease activity and US score in group 2 (p=0.04). CONCLUSION: This study confirms that peripheral joint involvement represents an important clinical aspect in italian BD patients and US evaluation is useful for the detection of this aspect.

Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: report of an international committee on nomenclature.

PURPOSE: To present revised criteria for the diagnosis of Vogt-Koyanagi-Harada disease, a chronic, bilateral, granulomatous ocular and multisystem inflammatory condition of unknown cause. METHODS: Diagnostic criteria and nomenclature were subjects of discussion at the First International Workshop on Vogt-Koyanagi-Harada Disease on October 19-21, 1999, at the University of California, Los Angeles, Conference Center, Lake Arrowhead, California. A committee appointed by the workshop participants was charged with drafting revised criteria for Vogt-Koyanagi-Harada disease, based on discussions held during the conference. This article is the consensus committee report. RESULTS: New criteria, taking into account the multisystem nature of Vogt-Koyanagi-Harada disease, with allowance for the different ocular findings present in the early and late stages of the disease, were formulated and agreed upon by the committee. These criteria are based on additional knowledge and experience of experts in the field and are believed to reflect disease features more fully than previously published criteria. CONCLUSIONS: The revised definition of Vogt-Koyanagi-Harada disease, with expanded diagnostic criteria, will facilitate performance of studies involving homogeneous populations of patients, at various stages of disease, that address unanswered questions regarding treatment and disease mechanisms.

Long-term follow-up of anterior uveitis after cataract extraction and intraocular lens implantation.

PURPOSE: To assess whether cataract surgery and intraocular lens (IOL) implantation in patients with anterior uveitis interfere with the natural course of the ocular disease. SETTING: Tertiary care center at the University of Rome "La Sapienza", Rome, Italy. METHODS: Cataract extraction and IOL implantation were performed in 24 patients with uveitis-related cataract: 12 with Fuchs' heterochromic iridocyclitis (Group 1) and 12 with other types of anterior uveitis (Group 2). The mean follow-up in the 2 groups was 33.6 months and 24.8 months, respectively. The number and severity (inflammatory score) of uveitis relapses in all patients over the same period were recorded. RESULTS: After surgery, the mean number and severity of uveitis relapses decreased: Group 1, from 1.83 +/- 1.90 (SD) to 1.00 +/- 1.21 and from 1.08 +/- 0.90 to 0.92 +/- 0.67, respectively; Group 2, from 2.74 +/- 3.44 to 1.25 +/- 1.71 and from 1.83 +/- 1.10 to 1.25 +/- 0.75, respectively (P = .046). There were no statistically significant between-group differences. In Group 2, a trend toward worse visual rehabilitation was seen; this was significantly different from the result in Group 1 (P = .018) because of preoperative optic nerve damage, macula disease, or both. CONCLUSION: Cataract surgery and IOL implantation did not negatively influence the natural course of uveitis in patients with Fuchs' heterochromic iridocyclitis. Correct surgical timing, selection of cases, and adequate anti-inflammatory therapy may promote similar results in patients with other types of anterior uveitis.

Bilateral trochlear nerve palsy associated with cryptococcal meningitis in human immunodeficiency virus infection.

This is the report of a case of bilateral trochlear nerve palsy secondary to cryptococcal meningitis in a 34-year-old woman with acquired immune deficiency syndrome. Based on clinical and neuroradiologic findings, it is concluded that in the present case, a postinflammatory shrinking of the arachnoid has stretched the fourth cranial nerves at their point of emergence from the dorsal surface of the brainstem.

The lactoferrin tear test in the diagnosis of Sjögren's syndrome.

PURPOSE: To assess the sensitivity and specificity of the lactoferrin tear test (LTT) in the diagnosis of keratoconjunctivitis sicca due to Sjögren's syndrome (SS), comparing it with the other lacrimal tests and with immunological tests. METHODS: 25 patients suffering from SS (24 women and 1 man, median age 51.5 years, s.d. 16.3); control group: 20 patients with various kinds of conjunctivitis without dry eye. Tests in both groups: Schirmer I (ST), BUT, ferning test, lactoferrin immunoassay, fluorescein and Rose Bengal staining. Immunological tests: serum titers of anti-nuclear (ANA), anti-DNA, anti-ENA (SS-A, SS-B, RNP, FR) antibodies. In the patients with SS, labial salivary gland biopsy was also performed. RESULTS: LTT had a specificity of 95% and a sensitivity of 72%, compared to 85% and 64% for the Schirmer I test. The ferning test has the highest sensitivity (92%), and none of the cases positive to BUT was negative to the ferning test. The combination of LTT and ferning test gave a value of 78% compared to 70% with ST. The correlations between positive LTT and positive ANA, SS-A and labial biopsy were respectively 83%, 67% and 80%, as against 67%, 50% and 65% for the Schirmer I test. CONCLUSIONS: In our study, LTT showed very high specificity, good sensitivity particularly when combined with qualitative tear tests, and a good correlation with the immunological and bioptic tests for SS. Since it is easy to perform, in our opinion LTT can be included in the diagnostic routine for keratoconjunctivitis sicca in SS.

Uveitis in children.

Uveitis is diagnosed more often in adults than in children but in children the prevalence of chronic inflammation and the difficulty of an early diagnosis may worsen the visual prognosis. The different incidence of the presumed or defined etiologies in the various ages is probably responsible for the better classification of uveitis in childhood (71% versus 55% in adult patients). Juvenile rheumatoid arthritis is the most common identifiable etiology of pediatric anterior uveitis (28%). However, its long-term prognosis is not satisfactory and ophthalmic surveillance protocols are necessary especially for ANA and HLA DR11 positive girls with the pauci articular form of the disease. Intermediate uveitis is idiopathic in 98% of the cases and accounts for 20-26% of all pediatric uveitis; cystoid macular edema in these patients is the leading cause of visual impairment. Toxoplasmosis is the most frequent cause of posterior uveitis (43%) and of uveitis in childhood (12%); involvement is bilateral in 50-60% of cases. Diffuse uveitis forms are uncommon in children, but their course is particularly severe. They may either be the natural evolution of a posterior uveitis or the presenting sign of a systemic disease typical of the third decade of life, such as sarcoidosis, Behçet's disease, Vogt-Koyanagi-Harada's disease. Masquerade syndromes including retinoblastoma, leukemia and lymphoma, intraocular foreign body, retinitis pigmentosa may contribute to confusion in the differential diagnosis of inflammatory disease of the uveal tract in childhood.

Relapsing bilateral uveitis and papilledema in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease).

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a non-hereditary histiocytic proliferative disorder affecting young people, with extranodal manifestations in 28-43% of cases. Eye involvement is infrequent. Lymphoproliferation in the soft tissues of the orbit and in the lids has been reported in 12% of cases but intraocular involvement is rare. We describe the case of a 12-year-old boy affected by Rosai-Dorfman disease with bilateral relapsing uveitis and papilledema that appeared four years before the onset of lymphadenopathy.

Magnetic resonance imaging of the parotid glands and lip biopsy in the evaluation of xerostomia in Sjögren's syndrome.

Magnetic Resonance Imaging (MRI) of the parotid glands was performed in 23 patients with dry mouth. Each patient underwent lip salivary gland (LSG) biopsy and complete clinical and immunological assessment. MRI showed a quite specific nodular pattern in the parotid glands of patients with Sjögren's syndrome (SS), especially those with severe histologic abnormalities in LSG. However no significant correlation could be detected between MRI score and both LSG biopsy class and immunological abnormalities. MRI of the parotid glands can be regarded as a useful noninvasive procedure with high positive predictive value for the evaluation of the salivary component in SS.

Clinical serological correlations in the evaluation of Sjögren's syndrome.

Fifty-six subjects (46 females and 10 males) with signs of Sjögren's Syndrome (SS) have been evaluated for clinical and serological parameters. In 12 subjects such clinical signs were associated with a definite connective tissue disease (secondary SS): Rheumatoid Arthritis (2 cases), Systemic Lupus Erythematosus (4 cases), Progressive Systemic Sclerosis (4 cases), Essential Mixed Cryoglobulinemia (2 cases). All patients underwent a lip biopsy which was evaluated according to the Chisholm-Mason scale. Six out of 56 subjects (10.7%) had a normal salivary gland with few lymphocytes (class I); 5 (10%) had a scattered lymphoid infiltrate containing less than 1 focus/4 mm2 (class II); 36 (64.3%) had one focus/4 mm2 (class III) and, finally, 9 (16%) had more than 1 focus/4 mm2 (class IV). The great majority (91%) of the patients had some abnormalities at the ophthalmological examination (Schirmer test, Break up times, van Bijsterveld score): even those with a class I lip biopsy. Xerostomia was present in 69% of the patients, xerophthalmia in 78%, arthralgias in 78%, parotid swelling in 19%, Raynaud's phenomenon in 16%, recurrent abortion in 9 patients. The prevalence of autoantibodies in the group of primary SS was high: ANA 43.2%, anti-Ha 40%, anti-Ro 12%, anti-phospholipids 52.2%, rheumatoid factor (RF) 45.7%. A decrease of lysozyme concentration in tears and saliva has been demonstrated and a correlation between level of lysozyme and lip-biopsy has been observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Retinal cotton-wool-like spots: a marker for AIDS?

The prognostic value of ocular manifestations and their correlation with immune changes in HIV-infected subjects (75 PGL, 23 ARC, and 17 AIDS) have been longitudinally studied with an average follow-up of one year (3 to 22 months). The most common ocular manifestations were retinal cotton-wool-like spots, observed in 58.8% of AIDS patients and in 76.9% of those with ocular involvement. Two of three ARC patients who showed cotton-wool-like spots developed PCP a few weeks after ophthalmoscopic examination. A close correlation between ocular changes and decrease of CD4+ lymphocytes was observed. In our opinion, these ocular manifestations are as useful an indicator as opportunistic infections or AIDS-related neoplasias in the prognosis of HIV infection.