Blindness in a Sri Lankan woman with bilateral breast lumps: a case report.

BACKGROUND: Granulomatosis with polyangiitis is a rare multisystemic autoimmune disorder predominantly affecting the upper and lower respiratory tracts and the kidneys, and rarely affecting other organ systems. Tuberculosis can mimic the presentation of granulomatosis with polyangiitis, and both can occur simultaneously in the same patient. Here we report what we believe to be the first case of concurrent granulomatous breast lesions and hemorrhagic retinal angiopathy in a Sri Lankan woman with refractory granulomatosis with polyangiitis complicated by probable tuberculosis. CASE PRESENTATION: A 48-year-old Sri Lankan Moorish woman presented with a 6-month history of ulcerating bilateral breast lumps, a 3-month history of non-healing painful ulcers on the palate, and sudden bilateral painless loss of vision. Retinoscopy confirmed left-sided retinal hemorrhages and bilateral panuveitis. An examination of her respiratory system showed bilateral coarse crepitations. Histologic examination of the palatal and breast lesions showed chronic granulomatous inflammation. Her levels of immune markers were elevated but her renal function was normal. Chest radiography showed bilateral mid-zone and lower-zone infiltrates with cavitation and small pleural effusions. Her serum proteinase 3 anti-neutrophil cytoplasmic antibody titer and the level of adenosine deaminase in her pleural fluid were significantly elevated. She was diagnosed with generalized granulomatosis with polyangiitis complicated with probable pulmonary tuberculosis, and was started on methylprednisolone and cyclophosphamide pulse therapy with anti-tuberculous treatment. She later developed cerebral vasculitis, indicating refractory disease, and was treated with second-line rituximab with excellent response. CONCLUSION: Proteinase 3 anti-neutrophil cytoplasmic antibody may be a valuable diagnostic marker in patients with atypical symptoms of granulomatosis with polyangiitis or in the presence of probable tuberculosis. Retinal vascular angiopathy needs to be diagnosed and treated early to prevent the development of complete blindness. Concomitant cytotoxic and anti-tuberculous treatments may be safe and effective in patients with simultaneous refractory disease with probable tuberculosis.

Vasculitis-like hemorrhagic retinal angiopathy in Wegener's granulomatosis.

BACKGROUND: Granulomatosis with polyangiitis, also known as Wegener's granulomatosis, is a chronic systemic inflammatory disease that can also involve the eyes. We report a case of massive retinal and preretinal hemorrhages with perivascular changes as the initial signs in granulomatosis with polyangiitis (Wegener's granulomatosis). CASE PRESENTATION: A 39-year-old Caucasian male presented with blurred vision in his right eye, myalgia and arthralgia, recurrent nose bleeds and anosmia. Fundus image of his right eye showed massive retinal hemorrhages and vasculitis-like angiopathy, although no fluorescein extravasation was present in fluorescein angiography. Laboratory investigations revealed an inflammation with increased C-reactive protein, elevated erythrocyte sedimentation rate and neutrophil count. Tests for antineutrophil cytoplasmic antibodies (ANCA) were positive for c-ANCA (cytoplasmatic ANCA) and PR3-ANCA (proteinase 3-ANCA). Renal biopsy demonstrated a focal segmental necrotizing glomerulonephritis. Granulomatosis with polyangiitis (Wegener's granulomatosis) was diagnosed and a combined systemic therapy of cyclophosphamide and corticosteroids was initiated. During 3 months of follow-up, complete resorption of retinal hemorrhages was seen and general complaints as well as visual acuity improved during therapy. CONCLUSION: Vasculitis-like retinal changes can occur in Wegener's granulomatosis. Despite massive retinal and preretinal hemorrhages that cause visual impairment, immunosuppressive therapy can improve ocular symptoms.

Repeated exacerbations of ocular inflammation with vitreous hemorrhage in a patient with HLA-B27 associated uveitis.

HLA-B27 associated uveitis is characterized by recurrent alternating acute unilateral attacks of intraocular inflammation in the anterior chamber. The aim of this study was to report an unusual case of repeated exacerbations with vitreous hemorrhage in HLA-B27 associated uveitis. Thirty four-year-old man was diagnosed as HLA-B27 associated uveitis in his right eye. He showed repeated exacerbation of ocular inflammation with retinal vein dilation and small retinal hemorrhage following vitreous hemorrhage. Fluorescein fundus angiography a week before the appearance of vitreous hemorrhage showed no neovascularization. Oral prednisolone administration was started from 40mg/day with gradual tapering. About 3 weeks after the onset, most of the vitreous hemorrhage disappeared and visual acuity was improved to 20/20. Through the decreased vitreous hemorrhage, Weiss ring was detected later. The vitreous hemorrhage found in this patient is a severe exacerbation, and might be a consequence of the vitritis that leads to posterior vitreous detachment.

[Bilateral central retinal vein occlusion associated with moderate acute rejection of a cardiac graft]. / Oblitération bilatérale de la veine centrale de la rétine sur un rejet aigu léger de greffe cardiaque.

We report a case of bilateral central retinal vein occlusion following moderate acute rejection of a cardiac transplant. A 27-year-old man was admitted for sudden bilateral decreased vision due to bilateral central retinal vein occlusion. Visual acuity was 20/63 in the right eye and 20/25 in the left eye. This patient had undergone a heart transplant 6 months before and had presented with moderate acute rejection for a few days. The moderate acute rejection phenomenon includes inflammatory lymphocyte infiltrates, reflecting persistent immune response activation. Moderate acute rejection of a cardiac transplant graft might cause a bilateral central retinal vein occlusion.

HLA typing in patients of Eales disease.

OBJECTIVE: To determine any predisposition of haplotypes with Eales disease. DESIGN: A case control study. PLACE AND DURATION OF STUDY: This study was started in February 2002 and data collected till April 2003 at Eye Department of Military Hospital, Rawalpindi. PATIENTS AND METHODS: The frequency of HLA antigens both class-I and II by complement dependent standard lymphocytotoxicity test was studied in 32 patients of Eales disease (group-I) and 32 age and gender matched normal persons as controls (group-II). Both patients and controls underwent complete ocular and clinical examination and were followed up for one year. RESULTS: Mean age was 30.8 years. HLA DR3 was found in 20 patients of group-I and none in group-II. HLA types A1, B8, B5 (51) and DR 15 (2) were found in 12 out of 32 patients of eales disease and none in controls. HLA DQ2 and DR52 was found in 28 cases of group-I as compared to 18 cases of group-II (p = .005). CONCLUSION: HLA phenotypes HLA DR3, A1, B8, B5 (51) and DR 15 (2) occurred in majority of cases of Eales disease, whereas these were not found in controls which was statistically significant. Similarly, HLA DQ2, DR52 and Bw6 was found in higher frequency in Eales patients and thus strongly associated with it. We conclude that certain HLA haplotypes have a possible predilection for Eales disease.

[Eales disease and tuberculous allergy]. / Maladie de Eales et allergie tuberculinique.

The authors report the case of a 25-year-old woman who presented unilateral Eales disease associated with biologically confirmed pulmonary tuberculosis. The patient, from a family with a history of tuberculosis, showed a painless and abrupt decrease in visual acuity of the right eye with venous and arterial vasculitis. Fluorescein angiography confirmed the existence of peripheral ischemia and vascular abnormalities. The biological exam showed a positive PPD and the BK was isolated. Antibiotic and corticosteroid drugs were used in the treatment. The clinical, immunopathological and therapeutic aspects of this disease are discussed.

[Retinal vasculopathy associated with Berger's IgA nephropathy]. / Retinale Vaskulopathie assoziiert mit Bergers IgA-Nephropathie.

AIM: Ophthalmological complications associated with Berger's IgA nephropathy comprise scleritis, episcleritis, keratoconjunctivitis as well as anterior uveitis. We present a new association of IgA nephropathy with a retinal vasculopathy. METHODS: Presentation of two clinical cases. RESULTS: Two patients presented with hematuria and epistaxis associated with a retinal vasculopathy characterised by teleangiectasies, capillary occlusion with retinal hemorrhages, neovascularisations and macular edema with decreased visual acuity. Fluorescein angiography showed zones of non-perfusion as well as vasculitic changes. A general medical exam revealed a normal arterial pressure but a slightly elevated creatinine. Immunological investigations for the presence of antibodies showed no positive results. Renal biopsy demonstrated mesangial proliferations with diffuse deposits of IgA. Over the course of a 2 year follow-up some of the retinal changes regressed under treatment with cortisone and visual acuity returned to normal. The teleangiectasies showed no progression. CONCLUSION: Berger's IgA nephropathy can be associated with a retinal vasculopathy which may be due to local deposition of IgA immune complexes in the retinal vessels.

[Retinal vasculitis and antiphospholipid antibodies]. / Retinale Vaskulitis und Anti-Phospholipid-Antikörper.

Antiphospholipid antibodies (APAb) are often found in systemic lupus erythematosus (LE) (secondary antiphospholipid syndrome), leading to arterial or venous thrombosis. In primary antiphospholipid syndrome other LE-associated symptoms are not detectable. We present the clinical course and therapy of three patients with this rarely reported disease, discussing immunopathology and therapy. Three young patients (28, 29 and 31 years) were seen at the university eye clinic, presenting severe occlusive vasculitis with vitreal haemorrhages and/or thrombosis. The first patient did not respond to any therapy (various immunosuppressives, plasma-pheresis, laser coagulation) and ended up with defective light perception in both eyes. The second patient also had light perception as final outcome in spite of acetylsalicylic acid, steroids and immunosuppression. The third patient established complete reperfusion on an arteriovenous occlusion after haemodilution and acetylsalicylic acid. Antibodies directed against phospholipids interfere with the blood clotting system in many ways (activation and aggregation of thrombocytes, endothelial function, coagulation cascade). The optimal therapy of this severe disease is unclear, suggesting that acetylsalicylic acid seems to be important, while the effect of immunosuppression or steroids is uncertain.

[Acute retinal necrosis syndrome. Lausanne cases, review of the literature and new physiopathogenetic hypothesis]. / Syndrome de nécrose rétinienne aiguë (ARN/BARN/PORN). Casuistique lausannoise, revue de la littérature et nouvelle hypothèse physiopathogénique.

PURPOSE: The purpose of this study was to analyse our ARN-patients, perform an extensive review of the literature, suggest a physiopathogenic hypothesis for the disease that should influence the therapeutical approach of these cases. PATIENTS AND METHODS: From 1985 to 1993, 15 HIV-negative cases of ARN were seen in our clinic. Eleven cases were analysed prospectively: the herpetic agent involved in each case was searched for by the determination of intraocular specific antibody production and a complete immunological work-up was performed. RESULTS: Our collective included 4 cases of bilateral ARN (BARN). In 9 cases the clinical presentation was that of a typical ARN, in 3 cases ARN was of the "mild-type" and in 3 cases lesions were multifocal involving initially the posterior pole resembling the clinical picture of PORN (progressive outer retinal necrosis). The viral agent was varicella-zoster virus in 7 cases, herpes simplex virus in 3 cases, cytomegalovirus in 1 case and was not determined in 4 cases. In the 216 published cases a state of immunodepression was found in 15.7%. In our group with systematic immunological work-up this rate was 46%. CONCLUSION: This is in support of the thesis that an immune dysfunction is probably at the origin of ARN. We therefore suggest to avoid to add systemic steroids to the specific antiviral therapy but to treat inflammation by periocular steroids.

[Antiphospholipid antibodies and occlusive vascular disease]. / Anti-fosfolipidantistoffer og okklusiv karsygdom.

The antiphospholipid syndrome is characterized by venous and/or arterial thrombosis, recurrent foetal loss, thrombocytopenia and the presence of antiphospholipid antibodies. This study presents three case reports, which emphasize the multiform clinical features of the syndrome: one case of uncomplicated myocardial infarction in childhood and two prolonged, clinically elusive case histories, both with fatal outcome due to acute, multifocal, non-inflammatory vascular occlusion. It is important to distinguish between this syndrome and various vasculitis syndromes, although consensus on therapy for the antiphospholipid syndrome has not yet been achieved.

[Retinochoroidopathy in a patient with seropositive trichinosis]. / Retinochorioidopathie bei einem Patienten mit seropositiver Trichinose.

A 28 years old butcher contracted a systemic trichinosis after fasting sausage. Twelve days after onset of systemic symptoms he developed a hemorrhagic maculopathy in his left eye. In addition, on both eyes there were focal atrophies of the retinal pigment epithelium in the mid-periphery. We believe a causal connection of the maculopathy with his serologically proven trichinosis as a retinal involvement is well known in trichinosis and histologically demonstrated by Herrenschwand (1928). To our knowledge this is the first case of hemorrhagic maculopathy in trichinosis.

Autoimmune encephalomyelitis and hemorrhagic retinal disease in neonatal, infant, juvenile, and adult monkeys.

Severe (24/24) and lethal (22/24) autoimmune encephalomyelitis was regularly induced in rhesus monkeys of all age groups from newborns to adults by a single injection of guinea pig spinal cord in complete Freund's adjuvant. Age dependency of the encephalomyelitis was manifested by a delayed onset and prolonged course in newborn monkeys. A hemorrhagic retinopathy usually accompanying the earliest CNS symptoms was observed in most of these monkeys. The most severe lesions were predominant in the cerebellum and brainstem of neonates, and in the cerebral hemispheres of older animals.

Missing evidence for HLA antigen association with Eales' disease, chorioretinitis, central serous retinopathy, and malignant choroidal melanoma.

Patients with Eales' disease, chorioretinitis, central serous retinopathy, or malignant choroidal melanoma were tested for HLA antigen deviation. When corrected p values (pc) are used, the first three disorders did not show any significant deviation, whereas a significant increase of HLA-Aw32 (pc = 0.026) was found in the malignant melanoma group. For conclusive evidence the latter finding needs confirmation by analysis of a greater number of patients with this disorder.