SESSION TYPE: Miscellaneous Global Case Report PostersPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

Rheumatoid arthritis has multiple affections that may include the lungs. The later are associated with disease stage and progression as well. In his report, we present the case of a male patient with long standing, veneer treated, rheumatoid arthritis presenting with a rather challenging pulmonary complication.

CASE PRESENTATION:

A 64 years old bedridden male was admitted to the hospital after 5 days of chills, shortness of breath and persistent cough with moderate yellow sputum and left base pleuritic pain. Medical history was remarkable for alcoholism and heavy smoking that quit more than 10 years ago. He had history of rheumatoid arthritis (RA) for more than 20 years never treated, and arterial hypertension (HTN). On admission his temperature was 36 C, blood pressure 87/55 mmHg , pulse 98 bpm, and respiratory rate 22/min with O2 sat at 96%. The white blood cell count was 6.38 (42% neutrophil, 0% band, lymphocyte 38%, monocytes 4.5% and eosinophils 11.8%); the hemoglobin levels was 8.1mg/dL , ESR=24mm, HIV negative, and VDRL negative. The PaO2 at room air was 100 mmHg. A chest X-rays on admission showed bilateral basal infiltrates and left pleural effusion. Patient started on IV antibiotics with minimal clinical response, gram stain of the sputum revealed gram positive cocci, three blood cultures obtained on admission yielded negative. On day 9, patient developed hemoptysis, night fever and sweats. Chest CT scan on day 10 revealed right upper lobe cavity, bilateral emphysematous changes and large loculated fluid collection (abscess). PPD test negative, and second chest CT on day 20 demonstrated multiple irregular shaped cavity lesions at upper lobes, persistent consolidation at the lower lobe with empyema and abscess, and mediastinal adenopathy.

DISCUSSION:

A cavity has been defined in the radiology literature as "gas filled space within a zone of pulmonary consolidation or within a mass or nodule produced by the expulsion of necrotic part of the lesion via the bronchial tree"(1). Cavity lesion is the result of any pathological processes including suppurative necrosis (e.g., pyogenic lung abscess), caseous necrosis (e.g, tuberculosis), ischemic necrosis (e.g, pulmonary infarction), cystic dilatation of the lung structure (e.g, PCP pneumonia), or displacement of lung tissue by cystic structure (e.g, Echicoccocus)(1). Many autoimmune diseases can affect the lung, but cavitation is relatively uncommon in most of these diseases. Rheumatoid arthritis is also commonly associated with pulmonary abnormalities, but cavities due primarily to rheumatoid arthritis are rare (1,2). Lung cavities in patients with rheumatoid arthritis often represent infection or carcinoma, thus, aggressive diagnostic evaluation is warrant for new cavitary lesions in these patients (1-3).

CONCLUSIONS:

Rheumatoid arthritis (RA) is a generally progressive systemic autoimmune process characterized by chronic symmetrical erosive synovitis. Nonarticular manifestations of RA include subcutaneous nodules, vasculitis, pericarditis, mononeuritis multiplex, and episcleritis (4,5). In rare cases, rheumatoid nodules may appear in the lung and cavitate, presumably due to ongoing vasculitis with ischemic necrosis (1-3).1) Cavitary pulmonary diseases; l. Beth Gadkowski and Jason E. Stout: Clinical Microbiology Review. 2008 April; 21(2): 305-333.2) A 64-year-old woman with a history of rheumatoid arthritis and new cavitary lung lesions. Boé DM, Schwarz mi, Groshong SD, Maloney JP. Chest. 2007 dec; 132(6):2046-52.3) Cavitary lung lesion in a patient with Sjögren's syndrome. Pérez-Castrillón JL, González-Castañeda C, Del Campo F, González JI, Martín-Escudero JC,DISCLOSURE: The following authors have nothing to disclose: Rafael Trinidad Hernandez, Hector Guerra-Garofalo, Ivonne Jimenez-Velazquez, Mark VergaraNo Product/Research Disclosure InformationUniversity of Puerto Rico, School of Medicine, San Juan, Puerto Rico.

Vision threatening ocular inflammation may occur in patients with any of the acquired connective tissue disorders and vasculitic diseases. Additionally, the ocular inflammation may be the presenting manifestation of the disease, which leads the patient to seek medical care. Other manifestations of the potentially lethal disease may be subtle or absent, presenting the thoughtful ophthalmologist with the opportunity to make life saving discoveries. Necrotizing scleritis, peripheral ulcerative keratitis, and retinal vasculitis are the ocular findings which should prompt the ophthalmologist to initiate very aggressive measures aimed at discovering any evidence of extra-ocular abnormalities, laboratory or otherwise. Appropriate therapy will be sight saving and may be life saving.

Acute myositis is the second most common component of non-specific orbital inflammation. We will describe its clinical features and natural history. This is a retrospective study of 10 cases. The diagnosis of acute myositis was based on clinical and imaging criteria. Our study includes five men and five women. The average age was 35.8 years (17-59 years). Clinical symptoms were: pain increased on eye movement (10/10), diplopia (4/10), proptosis (6/10), visual loss (3/10), lid edema (6/10), conjunctival hyperemia (7/10), anterior scleritis (2/10), episcleritis (2/10), chemosis (4/10), upper lid retraction (1/10), limitation of eye movement (3/10), fundus abnormalities (2/10). Imaging showed thickening of one or more extraocular muscles (10/10). Recovery was complete with anti-inflammatory therapy in six patients. Three patients experienced recurrence, and one patient had a clinical rebound upon tapering the treatment. Acute myositis can be defined by pain on eye movement, signs of inflammation, and extraocular muscle thickening on imaging. If the clinical presentation is typical, histopathological analysis can be deferred but remains necessary in cases of poor response to treatment, chronic duration or suspicion of tumor infiltration. The diagnosis of acute myositis may be suspected in the presence of consistent, well-defined clinical signs. Contiguous inflammation is often associated. Treatment is based on steroids or non-steroidal treatment anti-inflammatory therapy, administered alone or consecutively. Recurrences are frequent but do not alter the final prognosis.

Abstract

Purpose:

To report 2 cases of refractory idiopathic scleritis treated with rituximab.

Design:

Interventional case series.

Methods and Results:

Case 1: A 54-year-old woman, presented with anterior idiopathic scleritis in her right eye. Treatment with systemic steroids was not effective. Intravenous cyclophosphamide was discontinued after an adverse event. Case 2: A 43-year-old woman presented with idiopathic posterior scleritis in her left eye. Initial treatment with steroids was ineffective. In both cases, long-lasting remission without further relapses was achieved after 4 weekly doses (375 mg/m(2)) of rituximab.

Conclusion:

Rituximab was found to be an effective treatment for refractory idiopathic anterior and posterior scleritis.

To report the challenges in management in a case of scleritis with extensive staphylomas with secondary glaucoma. A 35-year-old one eyed female, a known case of scleromalacia perforans, presented with raised intraocular pressure on maximum medical treatment. She underwent successful Ahmed glaucoma valve surgery with exposure of the implant following a repeat episode of necrotizing scleritis after 3 months. Management of eyes with scleritis and secondary glaucoma can be challenging with unexpected complications and postoperative course.

We report an atypical presentation of Toxoplasma retinochoroiditis with associated scleritis in a young and immunocompetent patient. The diagnosis was done on the basis of Polymerase chain reaction of vitreous sample, and the clinical response to specific treatment. This case highlights the unusual presentation of ocular toxoplasmosis as scleritis.

PurposeTo describe the clinical and imaging presentation, pitfalls in the diagnosis of choroidal extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), as well as the therapeutic management and prognosis.MethodsA retrospective case review of nine choroidal MALT lymphomas was performed. Initial clinical presentation and imaging findings of these histologically confirmed cases of lymphoma were analyzed. Treatment methods, time to diagnosis, systemic work-up, and treatment prognosis were assessed.ResultsInitial presentation was essentially blurred vision. The features described on examination were: anterior and posterior scleritis, iridocyclitis, choroidal infiltration, and exudative retinal detachment. Fluorescein and indocyanine green angiography as well as ultrasonography and optic coherence tomography provided arguments in favor of the diagnosis. Biopsy sites included conjunctiva, Tenon's capsule, deep scleral tissue, episclera, lacrimal gland, and choroid. Treatment mostly consisted of a combination of chemotherapy and radiotherapy. The mean time to diagnosis was 12 months.ConclusionsOwing to the insidious onset of these tumors and their ability to simulate other conditions, the diagnosis is commonly delayed. The prognosis is generally good and treatment is effective in the case of localized lymphoma.Eye advance online publication, 19 April 2013; doi:10.1038/eye.2013.74.

This report describes the histopathological findings in a patient with Acanthamoeba sclerokeratitis (ASK). A 58-year-old patient with ASK underwent enucleation and sections of the cornea and sclera were subjected to histopathology and immunohistochemistry with monoclonal mouse antihuman antibodies against T cell CD3 and B cell CD20 antigens. Hematoxylin and Eosin stained sections of the cornea revealed epithelial ulceration, Bowman's membrane destruction, stromal vascularization, infiltration with lymphocytes, plasma cells, and granulomatous inflammation with multinucleated giant cells (MNGC). The areas of scleritis showed complete disruption of sclera collagen, necrosis and infiltration with neutrophils, macrophages, lymphocytes, and granulomatous inflammation with MNGC. No cyst or trophozoites of Acanthamoeba were seen in the cornea or sclera. Immunophenotyping revealed that the population of lymphocytes was predominantly of T cells. Granulomatous inflammation in ASK is probably responsible for the continuance and progression of the scleritis and management protocols should include immunosuppressive agents alongside amoebicidal drugs.