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VKC.pptx
- 1. VKC Presenter- DR SHAYRI PILLAI Liberia Eye Centre JFK Memorial Medical Centre L V Prasad Eye Institute November 13, 2019
- 3. Introduction » Vernal keratoconjunctivitis (VKC) is a chronic, bilateral, at times asymmetrical, seasonally exacerbated, allergic inflammation of the ocular surface, involving tarsal and⁄or bulbar conjunctiva » More common in children and young adults » Has an atopic background » First mentioned in the ophthalmic literature as conjunctiva lymphatica more than 150 years ago » More common in the summers than in the winters Kumar, Sunil Department of Ophthalmology, Mohammad Dossary Hospit al, Al Khobar, Saudi Arabia, Acta Ophthalmologica 2009
- 4. » The efficiency of school-aged children decreased profoundly because of the chronic and recurrent course » Although this is not usually a blinding disease, visual impairment may occur if the cornea is involved
- 5. Geographical distribution » VKC has a wide geographical distribution » Young males in dry and hot climates are primarily affected » It is more common in temperate zones of Mediterranean areas, central and west Africa, the Middle East, Japan, the Indian subcontinent and South America. » VKC cases are also seen in Western Europe, Australia and North America Kumar, Sunil Department of Ophthalmology, Mohammad Dossary Hospit al, Al Khobar, Saudi Arabia, Acta Ophthalmologica 2009
- 6. Demography » VKC starts before the age of 10 years » The earliest reported age of onset is 5 months (Ukponmwan 2003) » Resolves after puberty, usually around 4–10 years after onset (Bielory 2000; Leonardi 2002a) » The disease is more common among males, ratio varying from 4 : 1 to 2 : 1 ( Bonini et al. 2000) » After the age of 20 years, the male and female ratio becomes almost equal (Bielory 2000 Kumar, Sunil Department of Ophthalmology, Mohammad Dossary Hospit al, Al Khobar, Saudi Arabia, Acta Ophthalmologica 2009
- 7. Genetics and family history » No genetic predisposing factor has been identified for VKC » Predominance of VKC in Asia and Africa strengthens the possibility of a genetic predisposition » VKC is more common among individuals of Asian and African origin living in Sweden (Montan et al. 1999).
- 8. » Relationship between VKC and a particular genotype is not confirmed » Eosinophils in blood, tears and conjunctival scrapings » Expression of mediators and cytokines » Predominance of CD4 cells locally suggest that VKC may be a phenoty pic model of upregulation of the cytokin e gene cluster on chromosome 5q.
- 9. » Family history of allergic disorders such as – asthma – rhinitis – eczema – urticaria and – multiple atopic diseases reported in 49% of patient s suffering from VKC (Bonini et al. 2000). Kumar, Sunil Department of Ophthalmology, Mohammad Dossary Hospit al, Al Khobar, Saudi Arabia, Acta Ophthalmologica 2009
- 10. Associated conditions » Atopy is common among VKC patients – Asthma is the most common atopic disease seen among VKC patients » Keratoconus and acute hydrops – Due to excessive eye rubbing » Sex-hormone-related diseases such as – Gynaecomastia – Polycystic ovary syndrome – Mammary fibroadenoma – Adiposogenital dystrophy and – Autoimmune diseases were repo rted by 2 % of patie nts sufferi ng with V KC (Bonin i et al. 20 00)
- 11. Pathophysiology » VKC is characterized by infiltration of the conjunctiva by a variety of inflammatory cell types, especially eosinophils » Increased number of activated CD4+ T-lymphocytes, predominantly Th2, indicating that there is a hypersensitivity reaction to an unknown pathogen » Increased levels of inflammatory cytokines IL-3, IL-4, and IL-5 have also been demonstrated » Conjunctival papillae formation is related to fibroblast activation and production » Limbal conjunctival nodules are related to infiltration of inflammatory cells Hampton, Addis et Bennie H Jeng . Vernal keratoconjunctivitis,Clinical Ophthal mology 2018:12 119–123
- 12. » Cellular irregularities in patients with VKC » Injury to the superficial corneal epithelial layer and the basal epithelium and anterior stroma » Corneal nerves may be affected – decreased density – increased concentration of adjacent inflammatory cells Hampton, Addis et Bennie H Jeng . Vernal keratoconjunctivitis,Clinical Ophthal mology 2018:12 119–123
- 13. » Aberrations in the normal ocular surface microbiome may play a role in VKC » In a recent study, Staphylococcus aureus was more frequently isolated from the conjunctival specimens from patients with VKC, and may be a significant cause of exacerbations, while S. epidermidis was more frequently found in normal control patients. Hampton, Addis et Bennie H Jeng . Vernal keratoconjunctivitis,Clinical Ophthal mology 2018:12 119–123
- 14. Classification » Palpebral VKC involves the upper tarsal conjunctiva. » Associated with significant corneal disease as a result of the close apposition between the inflamed conjunctiva and the corneal epithelium » Limbal disease typically affects black and Asian patients » Mixed VKC has features of both palpebral and limbal disease. Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2016. Australia.P.145
- 15. Clinical features Symptoms » Intense itching » Lacrimation » Photophobia » Foreign body sensation » Burning and thick mucoid discharge » Increased blinking is common Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic Approach, 8th Ed. 2016. Australia.P.145
- 16. Signs Palpebral disease » Early-mild disease is characterized by » Conjunctival hyperaemia and » Diffuse fine papillary hypertrophy on the superior tarsal plate
- 17. » Macropapillae (<1 mm) have a flat-topped polygonal appearance focal inflammatory infiltrate s diffuse whitish inflammatory infiltra tes
- 18. Giant papillae (>1 mm) Mucus deposition between giant papillae Decreased disease activity is character ized by milder conjunctival injection and decreased mucus product ion
- 19. » Large (> 1 mm) papillae in VKC occur predominantly at the upper tarsus » Papillae that may attain a size of 7–8 mm are known as cobblestone papillae
- 20. » Limbal disease » Gelatinous limbal conjunctival papillae that may be associated with transient apically located white cellular collections (Horner–Trantas dots) In tropical regions, limbal disease may be severe
- 21. » Keratopathy is more frequent in palpebral disease and may take the following forms: » Superior punctate epithelial erosions associated with layers of mucus on the superior cornea
- 22. » Plaques and ‘shield’ ulcers
- 24. Grading of papillae » Bonini et al. (2000) graded the papillae on the upper tarsal conjunctiva or at the corneoscleral limbus as follows: » Grade 0: no papillary reaction » Grade 1+: few papillae, 0.2 mm widespread over the tarsal conjunctiva or around the limbus » Grade 2+: papillae of 0.3–1 mm over the tarsal conjunctiva or at the limbus » Grade 3+: papillae of 1–3 mm all over the tarsal conjunctiva or for 360o around the limbus » Grade 4+: papillae of more than 3 mm over the tarsal conjunctiva or gelatinous appearance at the limbus
- 25. Diagnosis » The diagnosis is clinical » Investigations are generally not indicated » Eosinophils may be abundant in conjunctival scrapings
- 26. Periodicity of disease » Important parameter to consideration while planning treatment » Two or three episodes in a year may be safely given short courses of mild steroids but in a child with chronic all year long disease, it may not be a good option to give chronic continuous steroid therapy.
- 27. » Intermittent disease periodicity is defined as inflammation free intervals of >2–3 months during which the patient is off medications – maximum of 3–4 episodes in a year, which remit on therapy » Chronic disease periodicity is defined as inflammation free intervals of <1 month during which the patient is off medications. – Patient has continuous ongoing inflammation, which possibly recurs on attempting to stop or taper therapy – Patient has chronic disease all throughout the year
- 28. Treatment Algorithm » Classify the disease severity into mild, moderate- intermittent, moderate-chronic, severe, and blinding subtypes for treatment Gokhale, Nikhil S Gokhale Eye Hospital, Mumbai, Maharashtra, India, Volume : 64 I ssue : 2 Page : 145-148 IJO 2016
- 29. Mild disease » Redness and itching » Congestion » Fine velvety papillae » No corneal involvement » Steroids should be avoided in the absence of any corneal involvement Rx Allergen avoidance (A) Lubricants (L) Antihistaminics (H) Mast cell stabilizers (M) Gokhale, Nikhil S Gokhale Eye Hospital, Mumbai, Maharashtra, India, Volume : 64 I ssue : 2 Page : 145-148 IJO 2016
- 30. Moderate disease (intermittent and chronic) » Corneal involvement: » Fine punctate erosions » Horner– Trantas dots » Limbal inflammation and » thickening of <6 clock hours In addition to ALHM Rx: intermittent Mild surface steroid s (Loteprednol) Rx Chronic Cyclosporine 0.5% Gokhale, Nikhil S Gokhale Eye Hospital, Mumbai, Maharashtra, India, Volume : 64 I ssue : 2 Page : 145-148 IJO 2016
- 31. Severe disease » Large active cobblestones » Coarse erosions or keratitis » Macroerosions » Severe limbal inflammation – >6 clock hours » Pannus » Postinflammatory scarring Rx Potent topical steroids + ALHM + 1% cyclosporine thera py Gokhale, Nikhil S Gokhale Eye Hospital, Mumbai, Maharashtra, India, Volume : 64 I ssue : 2 Page : 145-148 IJO 2016
- 32. » Blinding disease » Extremely active large cobblestones » Active shield ulcers, » Severe annular limbal inflammation, » Limbal stem cell deficiency – extensive conjunctivalization – limbal scarring – extensive corneal scarring Rx Potent steroid s + ALHMC Cyclosporine 2% e/d + tacrolimus 0.0 3% Gokhale, Nikhil S Gokhale Eye Hospital, Mumbai, Maharashtra, India, Volume : 64 I ssue : 2 Page : 145-148 IJO 2016
- 33. Patients with severe and blinding disease may also require surgical interventions: » Superficial keratectomy for shield ulcer plaques » Cryotherapy for refractory cobblestones » Cobblestone excision with or without mitomycin C » Amniotic membrane grafts » Reconstructive surgery ( limbal stem cell transplants) Gokhale, Nikhil S Gokhale Eye Hospital, Mumbai, Maharashtra, India, Volume : 64 I ssue : 2 Page : 145-148 IJO 2016
- 34. Differential diagnosis » Seasonal allergic conjunctivitis » Perennial allergic conjunctivitis » Atopic keratoconjunctivitis » Giant papillary conjunctivitis » Chlamydial infection » VKC may be differentiated from the above disorders by hypertrophic papillae in the tarsal form of VKC or Horner Trantas dots in the limbal form, or a combination of the two, along with no involvement of the eyelids.
- 35. Complications » Shield ulcers and plaques » Plaques form when inflammatory debris accumulates at the base of a shield ulcer » Limbal stem cell deficiency due to longstanding inflammation » Keratoconus and irregular astigmatism due to frequent eye rubbing » Steroid-induced glaucoma from frequent use of topical corticosteroid Hampton, Addis et Bennie H Jeng . Vernal keratoconjunctivitis,Clinical Ophthal mology 2018:12 119–123
- 36. Prognosis » Generally good and the disease is self-limiting with appropriate treatment » Despite an overall good prognosis, up to 6% of patients will develop vision loss due to complications associated with VKC » In patients studied, over half will continue to have symptoms after 5 years and the presence of giant papilla may indicate a worse prognosis Hampton, Addis et Bennie H Jeng . Vernal keratoconjunctivitis,Clinical Ophthal mology 2018:12 119–123
- 37. Take home message » VKC is a bilateral inflammatory disease that can cause visual loss » The diagnosis of VKC is largely clinical » Grading of the severity of disease and periodicity of disease can be very useful for deciding the appropriate line of management » Topical cyclosporine in higher concentrations and tacrolimus ointment are useful steroid-sparing agents » Severe, protracted cases of VKC remain a challenge to treat » Continue research is necessary to better understand the
- 38. Thank you! Excellence Equity Efficiency L V Prasad Eye Institute
Editor's Notes
- Western Europe (including the UK and Sweden),
- So far, no genetic predisposing factor has been identified for VKC but the predominance of VKC in Asia and Africa, along with the persistence of this predilection in migrated African and Asian populations, strengthens the possibility of a genetic predisposition.
- Relationship between VKC and a particular genotype not confirmed the constant and increased presence of eosinophils in blood, tears and conjunctival scrapings, the expression of a multitude of mediators and cytokines, as well as the predominance of CD4 cells locally suggest that VKC may be a phenotypic model of upregulation of the cytokine gene cluster on chromosome 5q. The cytokine gene cluster, throughits products like Interleukin (IL)-3, -4, -5 and granulocyte⁄macrophagecolony-stimulating factor (GM-CSF), regulate the prevalence of T helper cell type 2 (Th2), the growth and function of mast cells and eosinophils as well as the production of immunoglobulin (Ig) E in VKC (Bonini et al. 1995a).
- Atopy, defined as the presence of allergen-specific IgE antibodies, is common among VKC patients. One third of VKC patients have multiple atopic diseases (Bonini et al. 2000) Fifteen per cent of VKC patients were reported to have keratoconus; 6% of these developed hydrops (Iqbal et al. 2003). A higher incidence of keratoconus and acute hydrops among VKC has been ascribed to excessive eye rubbing (Cameron et al. 1989). Atopy is less common in limbal compared to tarsal VKC (Tuft et al. 1989)
- Palpebral VKC primarily involves the upper tarsal conjunctiva. It may be associated with significant corneal disease as a result of the close apposition between the inflamed conjunctiva and the corneal epithelium Limbal disease typically affects black and Asian patients. Mixed VKC has features of both palpebral and limbal disease.
- Symptoms consist of intense itching, which may be associated with lacrimation, photophobia, a foreign body sensation, burning and thick mucoid discharge. Increased blinking is common.
- diffuse velvety papillary hypertrophy on the superior tarsal plate
- Macropapillae (<1 mm) have a flat-topped polygonal appearance reminiscent of cobblestones; Focal or diffuse whitish inflammatory infiltrates may be seen in intense disease Reminiscent – similar to
- Thick mucus hyper-secretion with sticky mucous filaments, called ‘ropy discharge’, is a characteristic of VKC. Transient limbal or conjunctival yellow-white points or deposits, known as Horner–Trantas’s dots Progression to giant papillae (>1 mm) can occur, as adjacent smaller lesions amalgamate when dividing septa rupture Mucus deposition between giant papillae (Fig. 5.12E). Decreased disease activity is characterized by milder conjunctival injection and decreased mucus production (Fig. 5.12F).
- Epithelial macroerosions caused by a combination of epithelial toxicity from inflammatory mediators and a direct mechanical effect from papillae (Fig. 5.14B–D).
- Fig. 5.15 Keratopathy in vernal disease. (A) Early plaque; (B) plaque and shield ulcer; (C) subepithelial scarring following ulceration; (D) pseudogerontoxon and limbal papillae
- For example, a child who has two or three episodes in a year may be safely given short courses of mild steroids but in a child with chronic all year long disease, it may not be a good option to give chronic continuous steroid therapy.
- This would mean a maximum of 3–4 episodes in a year, which remit on therapy. Chronic disease periodicity is defined as inflammation free intervals of <1 month during which the patient is off medications. This would mean that patient has continuous ongoing inflammation, which possibly recurs on attempting to stop or taper therapy. Such a patient has chronic disease all throughout the year.
- It is usually possible to classify the disease severity into mild, moderate-intermittent, moderate-chronic, severe, and blinding subtypes Mild disease Patients will be symptomatic (redness and itching) and on examination havecongestion and fine velvety papillae but no corneal involvement. They should be treated with allergen avoidance (A), lubricants (L), antihistaminics (H), and mast cell stabilizers (M). Steroids should be avoided in the absence of any corneal involvement.
- Patients with corneal involvement in the form of fine punctate erosions, Horner– Trantas dots and focal limbal inflammation, and thickening of <6 clock hours are classified as a moderate disease. They require add-on therapy (in addition to ALHM) based on the periodicity of disease. In intermittent disease short pulses of mild surface acting steroids (e.g., loteprednol) can be given to tackle the recurrences. In chronic disease, long-term continuous therapy with topical 0.5% cyclosporine (C) is initiated and for persistent inflammation low-frequency mild steroids (typically once or alternate day once) can be added intermittently.
- These patients may have evidence of limbal deficiency in the form of pannus and postinflammatory scarring. Severe disease Patients with large active cobblestones, coarse erosions or keratitis, macroerosions, and severe limbal inflammation >6 clock hours are classified as a severe disease. limbal deficiency in the form of pannus and postinflammatory scarring. They require to be treated initially with the pulse of potent topical steroids (along with ALHM) and then maintained with chronic 1% cyclosporine therapy. Tacrolimus 0.03% ointment can also be used either transdermally or in the eye based on tolerance. Patients can require an additional maintenance therapy with low-frequency topical steroids (typically once or alternate day once).
- Patients with extremely active large cobblestones, active shield ulcers, severe annular limbal inflammation, limbal stem cell deficiency manifesting as extensive conjunctivalization, limbal scarring, and extensive corneal scarring and are the most difficult to treat.
- Differential diagnosis of VKC includes any of the chronic allergic conjunctivitis’s including seasonal allergic conjunctivitis, perennial allergic conjunctivitis, atopic keratoconjunctivitis, or giant papillary conjunctivitis, as well as chlamydial infection, especially in the early stages on the disease.11 VKC may be differentiated from the above disorders by hypertrophic papillae in the tarsal form of VKC or Horner Trantas dots in the limbal form, or a combination of the two, along with no involvement of the eyelids. Currently there is limited utility in testing for the diagnosis of VKC. Skin tests and IgE levels are rarely useful and may be negative in 50% of patients with VKC. In cases where the diagnosis is unclear, conjunctival scrapings showing eosinophilic infiltration may be beneficial in aiding the diagnosis.5
- The corneal epithelium acts as a barrier to circulating pathogens, but may become damaged in severe disease both due to trauma from upper tarsal papillae and a complex array of inflammatory molecules.34 This combination of repeated trauma and inflammatory milieu may then lead to shield ulcers and plaques They begin as punctate epithelial erosions which coalesce to form macroerosions which then develop into shield ulcers which can be self-limiting or develop further consequences such as bacterial keratitis
- The prognosis for VKC patients is generally good and the disease is generally self-limiting with appropriate treatment. Despite an overall good prognosis, up to 6% of patients will develop vision loss due to complications associated with VKC. In patients studied, over half will continue to have symptoms after 5 years and the presence of giant papilla may indicate a worse prognosis
- VKC is a bilateral inflammatory disease that can cause visual loss. Although little has changed in the diagnosis of VKC, which continues to be largely clinical, based on symptoms and clinical signs, newer therapies have been developed. This armamentarium of therapies continues to expand with increasing use of immunomodulators. Unfortunately, severe, protracted cases of VKC remain a challenge to treat. As such, continued research is necessary to better understand the complex nature of VKC and to develop more effective therapies.