A clinical case of recurrent episcleritis as the initial manifestation of granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA) is a type of small-sized blood vessel vasculitis that predominantly affects the upper airways, lungs and kidneys and associates with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Nevertheless, any organ of the body can be affected by GPA, including the eye. Occasionally, ocular involvement can be the initial manifestation, thus representing an essential clue for the physician in the early diagnosis of the disease. We present the case of a 53-year-old woman in whom recurrent episcleritis was the first sign of a multisystem disease. All further investigations led to the final diagnosis of GPA. The remission induction therapy chosen by the rheumatologist consisted of intravenous cyclophosphamide (CP) and methylprednisolone pulse-therapy, followed by oral glucocorticoids (GC). Based on the favorable clinical and paraclinical evolution, induction therapy was replaced by remission maintenance therapy. Azathioprine (AZA) was initiated and oral GC were continued, with dose tapering. Complete remission of episcleritis was observed. Abbreviations: GPA = granulomatosis with polyangiitis, EGPA = eosinophilic granulomatosis with polyangiitis, MPA = Microscopic polyangiitis, ANCA = Anti-neutrophil cytoplasmic antibodies, c-ANCA = ANCA to proteinase-3, p-ANCA = ANCA to myeloperoxidase, ELISAs = antigen-specific enzyme-linked immunosorbent assays, ENT = ear, nose, throat, CP = cyclophosphamide, NSAIDs = nonsteroidal anti-inflammatory drugs, AZA = azathioprine, GC = glucocorticoids.

A clinical case of orbital inflammatory pseudotumor as the primary expression of eosinophilic angiocentric fibrosis.

Eosinophilic angiocentric fibrosis (EAF) is an infrequent and slowly progressive disease, represented by fibroinflammatory lesions of unknown origin, which mainly involves the sinonasal structures and upper respiratory tract. Occasionally, it can affect the orbit and ocular adnexa causing symptoms such as proptosis, globe displacement and periorbital edema. In very rare cases, ocular manifestation as an orbital inflammatory pseudotumor can be the primary localization of the disease. Current literature proposes a relation between EAF and immunoglobulin G4-related disease spectrum. We describe the case of a 69-year-old man presented with antecedents of left periorbital edema, epiphora and retroocular pain. Examination showed a nonaxial proptosis, severe limitation in left eye adduction and lateral globe displacement. Orbital imaging revealed a left medial orbital mass with involvement of the inferior rectus and the medial rectus muscles. An orbital biopsy of the mass illustrated an inflammatory infiltrate with a notable eosinophilic component, "onion-skin appearance" of vessels and surrounding concentric fibrosis, highly suggestive of EAF. Further investigations showed a high expression of IgG4 and excluded other possible diseases. There was a favorable evolution of the orbital inflammatory pseudotumor following a 4-month treatment course with oral glucocorticoids. Abbreviations: EAF = Eosinophilic angiocentric fibrosis, CT = Computed tomography, MRI = Magnetic resonance imaging, GPA = Granulomatosis with polyangiitis, EGP = eosinophilic granulomatosis with polyangiitis, MPA = microscopic polyangiitis, ANCA = Anti-neutrophil cytoplasmic antibodies, Ig = Immunoglobulin.

Advances in the treatment of uveitis in patients with spondyloarthritis - is it the time for biologic therapy?

Spondyloarthritis (SpA) is a heterogeneous group of diseases that includes ankylosing spondylitis (AS), psoriatic arthritis (PsA), reactive arthritis (ReA), inflammatory bowel disease-associated spondyloarthritis (IBD-SpA), and undifferentiated spondyloarthritis (unSpA). This group of diseases shares several clinical, imaging, and genetic features; the integration of these diseases in the group of SpA is needed for an early diagnosis and a prompt treatment. Uveitis is the most common extra-articular manifestation of SpA. HLA-B27-associated acute anterior uveitis (AAU) is the most frequent form of uveitis encountered in the SpA group. The general prevalence of HLA-B27-associated AAU in the group of SpA is about 30% and the general prevalence of SpA in patients with HLA-B27-associated AAU is over 50%. There are several differences in the clinical picture and evolution of HLA-B27-associated AAU in patients with SpA and knowing this is very important for the best therapeutic decision. Tumor necrosis factor α (TNFα) is a very important mediator not only in the pathogenic mechanisms of SpA, but also in the immune reactions that characterize HLA-B27-associated AAU in SpA. There is much evidence of the role of TNFα in SpA and HLA-B27-associated AAU, multiple studies showing efficacy of anti-TNFα drugs not only on rheumatic manifestations but also on ocular involvement. Conventional therapy of HLA-B27-associated AAU with local or systemic glucocorticoids and immunosuppressive drugs (sulfasalazine, methotrexate, azathioprine, etc.) in order to diminish the ocular inflammation is associated with many side effects, some of them being very severe and even life threatening. Therefore, new treatments, especially biologic therapy with anti-TNFα drugs, open a new opportunity for the treatment of these patients. It is very important to emphasize that antibody anti-TNFα agents (infliximab, adalimumab, golimumab) may be more efficient than soluble receptors of TNFα (etanercept) in decreasing the risk of HLA-B27-associated AAU in patients with SpA. The aim of this review made by a group of ophthalmologists and rheumatologists with recent and fruitful experience regarding the anti-TNF treatment of uveitis in patients with SpA is to make the community of ophthalmologists aware of this biologic therapy and that it is the right time to use it. Abbreviations: AU = anterior uveitis; AAU = acute anterior uveitis; AS = ankylosing spondylitis; ASAS = Assessment of SpondyloArthritis Society; DBP = vitamin D binding protein; ESSG = European Spondyloarthropathy Study Group; HLA-B27 = human leukocyte antigen B27; IBD = inflammatory bowel disease; PsA = psoriatic arthritis; ReA = reactive arthritis; SpA = spondyloarthritis; TLRs = Toll-like receptors; TNFα = tumor necrosis factor α; unSpA = undifferentiated spondyloarthritis.