Inspection:
On ocular examination this aged patient has gross thinning of sclera with show of underlying uveal tissue. The uvea is covered with fibrous tissue and conjunctiva. There are large abnormal blood vessels crossing the areas of scleral loss.
There is no ocular congestion.
There is no peripheral ulcerative keratitis.
Also observe:
Deformed hands due to Rheumatoid arthritis.
Saddle Shaped deformity of nose (due to destruction of nasal cartilage) Wegener’s Granulomatosis & Relapsing Polychondritis
Ear deformity – Relapsing Polychondritis
Also check!
IOP
Corneal staining with fluorescein – Peripheral Ulcerative Keratitis
TBUT – Dry Eye associated with RA
Schirmer test – Dry eye associated with RA
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This condition is due to necrotizing scleritis without inflammation and occur in patients with long-standing rheumatoid arthritis.
Questions:
What types of scleritis do you know of?
Anterior scleritis diffusescleritis (most benign and 30% has underlying connective tissue disease) nodular scleritis (deep red to purple colour, immobile nodule) necrotizing scleritis (the most destructive form, 60% of affected patients develop ocular and systemic complications, 40% suffer loss of vision and 29% die within 5 year of onset usually due to complications of vasculitis), two types:
1. with inflammation
2. without inflammation
Posterior scleritis (least common, present with pain, proptosis, visual loss and restricted motility. Choroidal folds, exudative retinal detachment, papilloedema, and angle-closure glaucoma due to choroidal thickening may develop)
How common is perforation in cases of scleromalacia perforans?
It is quite rare.
What is the differential diagnosis?
Scleromalacia perforans
Senile Scleromalacia
Innocuous Scleral Hyaline Plaque
How bad is the visual acuity affected in SP?
Usually there is no gross effect on visual acuity.
What is the pathophysiology of SP?
It is type 3 hypersensitivity, with deposition of antigen/antibody complexes.
Three mechanisms have been described.
Activation of scleral fibrocytes and resorption of pericellular matrix,
infiltration of the scleral stroma by inflammatory cells,
prolonged local vaso-occlusion.
How does the disease progress?
The disease begins with Necrotic scleral plaquesn near the limbuts without vascular congestion, then these necrotic plaques coalesce and enlarge. Slowly, with the progression of scleral thinning, there is progressive exposure of underlying uvea.
What clinical Investigations would you advise?
As the condition is autoimmune associated with RA, SLE, WG, AND RPC, thus testing is conducted to single them out.
Complete blood count (CBC) with differential,
Erythrocye sedimentation rate (ESR) or C-reactive protein (CRP),
Serum autoantibody screen (including antinuclear antibodies, anti-DNA antibodies, Rheumatoid factor, antineutrophil cytoplasmic antibodies),
Urinalysis,
Syphilis serology,
Serum uric acid
Sarcoidosis screen.
How do you treat Scleromalacia Perforans?
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