A sore red eye with systemic involvement

• Published in: BMJ 2012-02, Vol.344 (feb24 1), p.e1121-e1121
• Main Authors: Shalchi, Zaid, Lightman, Susan L, Pusey, Charles D, Taylor, Simon R J
• Format: Article
• Language: English
• Subjects: Adult; Arthritis; Cyclophosphamide - administration & dosage; Eye; Glucocorticoids - administration & dosage; Granulomatosis with Polyangiitis - complications; Granulomatosis with Polyangiitis - diagnosis; Granulomatosis with Polyangiitis - drug therapy; Humans; Immunoglobulins; Immunosuppressive Agents - administration & dosage; Inflammation; Kidney diseases; Male; Multimorbidity; Myelosuppression; Neutrophils; Pathogenesis; Prednisolone - administration & dosage; Proteins; Reactive oxygen species; Respiratory system; Rheumatology; Scleritis; Scleritis - etiology; Systemic diseases; Systemic lupus erythematosus; Urinalysis; Urine
• ISSN: 0959-8138; 1468-5833; 1756-1833
• DOI: 10.1136/bmj.e1121

Long answer Necrotising scleritis is associated with a medical disorder in 39-50% of cases, particularly connective tissue disease or systemic vasculitis. 1 2 History taking, examination, and investigations should centre on a differential diagnosis of systemic lupus erythematosus; rheumatoid arthritis; Crohn's disease; or the antineutrophil cytoplasmic antibody (ANCA) associated vasculitides, including Wegener's granulomatosis. The ANCA test is usually positive in systemic Wegener's disease but can be negative, particularly if disease is limited to the orbit. 4 ANCAs may be present in other conditions (such as ulcerative colitis, ankylosing spondylitis, and Churg-Strauss syndrome), and a negative ANCA test does not exclude a diagnosis of Wegener's granulomatosis. 4 Direct immunofluorescence can distinguish different patterns of ANCA staining, and cytoplasmic ANCA (cANCA) staining is classic, whereas perinuclear ANCA (pANCA) staining is more commonly a feature of microscopic polyangiitis. ANCAs are present in 90-95% of patients with systemic Wegener's disease. 9 ANCAs directed against serum proteinase 3 lead to neutrophil activation, with resultant release of reactive oxygen species and proteolytic enzymes, which cause damage to the vascular endothelium and result in vasculitis. 10 Activation of the immune system leads to disease in small and medium sized vessels and causes multiorgan disease. Patients treated with cyclophosphamide must be monitored for serious treatment related toxicity, including bone marrow suppression and haemorrhagic cystitis. 12 Once remission is induced, introduce less toxic drugs such as azathioprine or methotrexate instead. 13 14 Taper steroids to 7.5 mg/day or less and continue treatment for at least one year.