Adult-onset Still's Disease (AOSD), often though as the adult variant of systemic juvenile idiopathic arthritis (JIA), has an incidence of 1-3 cases per 1 million. Cardinal manifestations include fever, arthritis, skin rash, sore throat, hepatosplenomegaly and lymphadenopathy. Prolongation in diagnosing this disease results from its similarity to infectious, malignant and rheumatic diseases and lack of biomarkers. Pulmonary arterial hypertension (PAH) is a rare pulmonary complication of AOSD, and we are aware of only six cases reported in literature to date. Here we present a patient with AOSD who has developed pulmonary hypertension as a complication. We report a case of AOSD complicated by PAH treated successfully with tocilizumab, a humanized monoclonal antibody to human interleukin (IL)-6 receptor. A Pubmed and Medline search for evidence of pulmonary hypertension in AOSD and use of IL-6 inhibition in management was performed. Data for this study was collected from the patient's chart records. No infectious or neoplastic cause of her disease was identified and after extensive diagnostic workup, the patient was diagnosed with AOSD fulfilling Yamaguchi criteria. After initiation of IL-6 therapy the patient was followed over time to monitor the hemodynamic changes in pulmonary vasculature. Following treatment with Tocilizumab, the patient showed dramatic improvement in her clinical symptoms and remains in remission, through combination of tocilizumab (8 mg/kg), methotrexate and prednisone. Improvement of systemic symptoms, right heart catheterization (RHC) findings and the VECTRA-DA score served as a measure of treatment response. Tocilizumab has been effective in demonstrating marked improvement in both the clinical and laboratory parameters. Tocilizumab is an effective novel treatment for AOSD with PAH. This is the first documented report of successful use of tocilizumab in AOSD patients presenting with PAH. Prospective comparative studies could help validate its efficacy and safety.
Objective. Rheumatoid arthritis (RA) patients are at increased risk of latent tuberculosis infection (LTBI) but there are no clear guidelines for LTBI screening with Tuberculin Skin Test (TST) or Quantiferon TB Gold testing (QFT-G). Methods. A retrospective study was conducted in a high risk, largely foreign-born, inner city, RA population. After screening 280 RA patients, 134 patients who had both TST and QFT-G testing performed during their initial evaluation were included. Results. Out of 132 RA patients included in our analysis, 50 (37.8%) patients were diagnosed with LTBI with either positive TST 42 (31.8%) or QFT-G 23 (17.4%). 15 (11.4%) were positive and 82 (62.1%) were negative for both tests. The agreement between TST and QFT-G was 73.5% (Kappa 0.305, CI = 95% 0.147–0.463, p = 0.081). Conclusions. There was low-moderate agreement (κ = 0.305) between TST and QFT-G. In the absence of clearly defined gold standard and limitations associated with both tests, we propose early screening with both tests for patients who need prompt treatment with BRMs. Patients who are not immediate candidates for BRM treatment may be safely and cost effectively screened with a two-step process: initial screening with TST and if negative, IGRA testing. Patients positive for either test should be promptly treated.
BackgroundAntiphospholipid syndrome (APS) is defined as the occurrence of venous or arterial thrombosis or pregnancy morbidity, in the presence of serological evidence of antiphospholipid antibodies (including IgM and IgG anticardiolipin antibodies, IgM and IgG anti-beta-2 glycoprotein I antibodies, or lupus anticoagulant). Whereas most patients with focal thrombotic events respond to anticoagulation, patients with multifocal occlusive disease are occasionally refractory to standard therapeutic interventions. Eculizumab, a monoclonal antibody that binds to complement protein C5 and prevents the conversion of C5 to C5a and C5b, may be an effective treatment for patients with APS. This is based on the data in pre-clinical APS models as well as anecdotal experience in humans. We administered eculizumab to four patients with severe refractory APS (two with primary APS and two with lupus and APS).MethodsFour patients with APS, unresponsive to conventional anticoagulant therapy, were treated with a loading dose of eculizumab followed by dosing every other week. Patients received appropriate vaccinations prior to treatment. The platelet count served as a surrogate marker of APS activity. During therapy, the patients were monitored for platelet counts, thrombotic events, and infections. Follow-up ranged from four months to one and one-half years, and remains ongoing.ResultsAt their lowest values, the patients had platelet counts of 35, 22, 18, and 85 (K/mL). One of the patients was steroid-dependent to maintain her platelet count at an adequate level. After initiation of eculizumab, the patient was able to taper steroids as the platelet count rose from a low of 35 to average counts of 100. The second patient's platelet count rose to over 200 from 22 within 10 days of receiving eculizumab. After one dose of eculizumab, the third patient's platelet count rose from 18 to 50 within four days, and has now stabilized at around 100. The fourth patient's platelet count rose to over 200. The increases in platelet counts were sustained other than during brief periods when therapy was delayed. During the treatment period, there were no new thrombotic events or infections in all four patients. One patient with long-standing renal disease expired after refusing dialysis.ConclusionsEculizumab has shown favorable results in our patients with refractory APS. Longer follow-up of these patients is needed in order to discern the effect on thrombosis. The role of complement inhibitors as a therapeutic intervention in this syndrome needs to be further elucidated.ReferencesShapira I et al. Induction of sustained remission in recurrent catastrophic antiphospholipid syndrome via inhibition of terminal complement with eculizumab. Arthritis Rheum 2012; 64(8):2719-2723.Comarmond C and Cacoub P. Antiphospholipid syndrome: from pathogenesis to novel immunomodulatory therapies. Autoimmun Rev 2013; 12(7):752-757.Barilla-LaBarca M, Toder K, Furie R. Targeting the complement system in systemic lupus erythematosus and other diseases. Clin Immunol 2013; 14...
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