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Year : 2002  |  Volume : 50  |  Issue : 4  |  Page : 317-319

Frosted branch angiitis associated with rapidly progressive glomerulonephritis.

Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh-160 012, India

Correspondence Address:
A Gupta
Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh-160 012
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Source of Support: None, Conflict of Interest: None

PMID: 12532498

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Simultaneous occurrence of frosted branch angiitis and immune-mediated rapidly progressive glomerulonephritis is reported. The two diseases possibly share a common immune mechanism. Patients of frosted branch angiitis should undergo complete systemic evaluation including renal function tests even if the patient is systemically asymptomatic.

Keywords: Frosted branch angiitis, rapidly progressive glomerulonephritis.

How to cite this article:
Gupta A, Narang S, Gupta V, Minz R, Sakhuja VK. Frosted branch angiitis associated with rapidly progressive glomerulonephritis. Indian J Ophthalmol 2002;50:317-9

How to cite this URL:
Gupta A, Narang S, Gupta V, Minz R, Sakhuja VK. Frosted branch angiitis associated with rapidly progressive glomerulonephritis. Indian J Ophthalmol [serial online] 2002 [cited 2020 Aug 4];50:317-9. Available from: http://www.ijo.in/text.asp?2002/50/4/317/14759

Frosted branch angiitis is a form of severe vasculitis. Whether it is a distinct clinical entity or a variety of disorders sharing the same fundus appearance is unclear.[1] In the past two decades, it has been reported to be associated with a variety of viral, bacterial, rickettsial, parasitic and systemic diseases like lymphoma, sarcoidosis, multiple sclerosis and Crohn's disease. However, most cases are idiopathic.[2][3][4] We report a case of frosted branch angiitis associated with idiopathic immune-mediated rapidly progressive glomerulonephritis (RPGN).

  Case report Top

A 28-year-old male presented to us with sudden profound decrease of vision in the right eye of three days' duration. Systemic history and systemic examination were unremarkable. On ocular examination, the visual acuity was hand movement close to face in the right eye and 6/6 in the left eye. Slitlamp biomicroscopy of the right eye showed 2+ cells in the anterior chamber and anterior vitreous. The fundus examination of the same eye revealed creamy, thick diffuse perivascular sheathing, scattered superficial retinal haemorrhages and parafoveal retinal oedema [Figure - 1]. There was no evidence of choroiditis or retinitis. Fluorescein angiography showed extensive fluorescein leakage from the veins and optic disc in the late dye transit phase ([Figure - 2] and [Figure - 3]). Left eye examination

was unremarkable and fundoscopy did not show any retinopathy. A diagnosis of frosted branch angiitis of the right eye was made. Systemic investigations including blood pressure, erythrocyte sedimentation rate, complete haemogram, peripheral blood film, rheumatoid factor, antinuclear antibody, Lupus erythematosis cells (LE), Anti neutrophilic-cryoplasmicantibodies (ANCA), C-reactive protein, toxocara titers, toxoplasma titres, Human Immunodeficiency Virus (HIV) titres, Herpes titres, Cytomegalo virus (CMV) titres, X-ray chest, Mantoux test, and Treponema pallidum haemagglutination test were within normal limits. However, the patient had haemoglobin of 9gm% and this could not be attributed to any definite cause. The renal function tests were not done at this time. The patient was started on oral prednisolone (1.5mg/kg) along with topical betamethasone 0.1% and atropine 1%. Within 3 weeks of starting treatment, there was resolution of perivascular sheathing, peripheral retinal exudates, decrease in superficial retinal haemorrhages and macular oedema [Figure - 4]. His best-corrected visual acuity after treatment improved to 6/60. The oral prednisolone was tapered. The resolution of vasculitis in the right eye was associated with arterial attenuation, occlusion of peripheral arterioles and disc pallor. The left eye was unremarkable at this time too.

At the follow-up examination 6 weeks later (while he was on a tapering dose of oral prednisolone, 30 mg/day) the best corrected visual acuity was 6/60 and 6/6 in right and left eye respectively. The vasculitis in the right eye had resolved. However, marked arteriolar attenuation was noted in the left eye, suggestive of hypertensive angiopathy. The same day, blood pressure recorded was 200/110 mm Hg. The renal functions were found to be markedly deranged (blood urea 52 mg% and serum creatinine 8 mg%) and the haemoglobin was 6.5 gm% Ultrasonography revealed a normal-sized kidney and the kidney biopsy showed 3-4 glomeruli with crescents, IgG and C3 deposition in the mesangium as well as along the capillary loops with patchy deposition of IgA and IgM [Figure - 5]. The findings were consistent with idiopathic immune-mediated crescentic RPGN. The patient underwent haemodialysis. He was awaiting a renal transplant.

  Discussion Top

Frosted branch angiitis is a relatively rare disease among young adults; it was first reported by Ito in 1976.[5] The disease is characterised by: severe sheathing of retinal vessels appearing like the frosted branches of a tree; acute visual disturbance associated with anterior chamber and vitreous inflammation; fundus fluorescein angiography that shows no occlusion or stasis of the sheathed vessels; fluorescein leakage from the sheathed vessels; and otherwise healthy patient and prompt response to corticosteroids.[6],[7] The present case fulfilled all the diagnostic criteria of frosted branch angiitis.

Frosted branch angiitis has so far not been reported to be associated with RPGN. The association of unilateral mild peripheral vasculitis and IgA nephropathy leading to acute renal failure has been noted previously.[8] In the past, both infective and immune-mediated aetiologies have been proposed for frosted branch angiitis. The acute onset and dramatic response to corticosteroids is suggestive of immune-mediated aetiology in our patient. In the present case, frosted branch angiitis appears to have preceded the onset of crescentic glomerulonephritis. However, the initial presentation of frosted branch angiitis was associated with unexplained anaemia. Though renal function tests were not done initially, and blood pressure readings were repeatedly normal, a concurrent onset of frosted branch angiitis and RPGN could not be entirely ruled out. At the 6-week follow-up the hypertensive retinopathy, high blood pressures and decreasing haemoglobin were suggestive of renal pathology. However, it is possible that the kidney pathology occurred simultaneously with ocular pathology but was missed during initial diagnosis. The renal pathology may have been masked by the use of systemic corticosteroids and flared up when corticosteroids were tapered. Kidney biopsy revealed deposition of immunoglobulins and compliment. Though similar changes could occur in the eye, there is no histologic evidence as yet of immunoglobulin deposition in the eyes of patients with frosted branch angiitis. RPGN is known to be associated with immune-mediated systemic diseases such as SLE, Good pasture syndrome, vasculitis (e.g. polyarteritis nodosa), Wegner's granulomatosis, Henoch Schonlein purpura, cryoglobulinemia, etc. However, no association of RPGN with eye disease has been reported so far.

We conclude that a patient of frosted branch angiitis should undergo complete systemic evaluation including renal function tests even if systemically asymptomatic.

The commonality of immune mechanisms as aetiology of frosted branch angiitis and RPGN makes it necessary to investigate this link more thoroughly.

  References Top

Kleiner RC. Frosted branch angiitis: Clinical syndrome or clinical sign? Retina 1997;1997:370-71.  Back to cited text no. 1
Biswas J, Raizada S, Gopal L, Kumarasamy N, Soloman S. Bilateral frosted branch angiitis and cytomegalovirus retinitis in acquired immunodeficiency syndrome. Indian J Ophthalmol 1999;47:195-97.  Back to cited text no. 2
Kleiner RC, Kaplan HJ, Shakin JL, Yannuzzi LA, Crosswell HH, McLean WC. Acute frosted retinal periphlebitis. Am J Ophthalmol 1988;106:27-34.  Back to cited text no. 3
Yasasaga JE, Davis J. Frosted branch angiitis with ocular toxoplasmosis. Arch Ophthalmology 1999;117:1260-61.  Back to cited text no. 4
Ito Y, Nakano M, Kyu N, Takeuchi M. Frosted - branch angiitis in child. Jpn J Clin Ophthalmol 1976;30:797-803.  Back to cited text no. 5
Watanabe Y, Takeda N, Adachi-Usami E. A case of frosted branch angiitis. Br J Ophthalmol 1987;71:553.  Back to cited text no. 6
Geier SA, Nasemann J, Klauss V, Kronawitter U, Goebel FD. Frosted branch angiitis in patient with acquired immune deficiency syndrome. Am J Ophthalmol 1992;113:203-05.  Back to cited text no. 7
O'Neil D, Harvey P, Longstaff S, Brown CB. Retinal vasculitis and uveitis in IgA nephritis. Eye 1994;8:711-13.  Back to cited text no. 8


  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]

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