Rodney Tehrani

Ocular Toxicity of Hydroxychloroquine

This review summarizes the current literature regarding the ocular complications of hydroxychloroquine. Hydroxychloroquine has been used since the 1950s for the treatment of various rheumatic and dermatologic diseases. Hydroxychloroquine can cause ocular toxicity, with the most serious being an irreversible retinopathy. At the present time, no “gold standard” exists for identification of the ocular toxicity prior to its development. This has led to controversy regarding the recommendations for ophthalmologic examinations for screening patients on hydroxychloroquine.

Refractory adult-onset still disease successfully treated with abatacept.

Adult-onset Still disease (AOSD) is an inflammatory condition of unknown etiology that responds to glucocorticosteroids and disease-modifying antirheumatic drugs, particularly methotrexate. However, disease refractory to conventional treatment has led to the reported use of biologic therapy including tumor necrosis factor α inhibitors (infliximab, etanercept, and adalimumab), anakinra, rituximab, and tocilizumab. We report the successful use of abatacept in the treatment of a patient with AOSD manifested by polyarthritis, rash, fevers, elevated liver function tests, and ferritin levels refractory to treatment with methotrexate and hydroxychloroquine. Remission has been maintained for 35 months with the addition of abatacept administered once monthly. There is evidence that T-cell activity plays an important role in the autoimmune activity of AOSD, and modulation of CD28 costimulation of T cells by abatacept has specific immunosuppressive actions that make it an appealing alternative therapeutic option for refractory AOSD.

Stiff-Person Syndrome: Persistent Elevation of Glutamic Acid Decarboxylase Antibodies Despite Successful Treatment With Rituximab

Stiff-person syndrome is a rare neurologic disorder characterized by fluctuating and progressive stiffness of axial and limb musculature. The high prevalence of autoantibodies and associated autoimmune diseases in patients with stiff person syndrome suggests that it may have an autoimmune etiology. We report a case of a 62-year-old man with diabetes who developed progressive stiffness of the hips and legs and elevated levels of antibody to glutamic acid decarboxylase. He had a partial response to both baclofen and diazepam, but could not tolerate the treatment because of somnolence. He eventually received 2 infusions of rituximab 1000 mg a week apart. The baclofen was discontinued and the diazepam was tapered. However, 6 months after the rituximab treatment, despite continued clinical improvement the patient had persistently elevated titers of antibody to glutamic acid decarboxylase. We postulate that rituximab was associated with clinical improvement through mechanisms other than antibody depletion.

Survival and extrapulmonary course of connective tissue disease after lung transplantation.

Background Connective tissue disease (CTD)–related lung dysfunction is a common cause of morbidity and mortality; however, few lung transplantations (LTs) are performed in this population secondary to uncertainty regarding the posttransplant survival, outcome, and management. Objectives The objectives were to evaluate the survival and the pulmonary and extrapulmonary courses of CTD after LT. Methods Survival outcomes of patients documented within the Organ Procurement and Transplantation Network who had undergone a LT for CTD were compared with those who underwent LT for chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). In addition, the pulmonary and extrapulmonary courses of the CTD were evaluated after LT. Results From 1991 to 2009, there were 284 documented LT in patients with CTD. Post-LT cumulative survival of patients with CTD was less than that for COPD through 5 years, with a difference that peaked at 1 year (72.7% vs. 83.1%, P < 0.001). When patients with CTD were compared with those with IPF, a difference was only noted at 1 year (72.7% vs. 77.7%, P = 0.049). There were no documented post-LT pulmonary recurrences of the CTD, and extrapulmonary flares of the CTD were rare (1 possible flare per 20.3 patient-years and 1 probable flare per 81.0 patient-years). Conclusions Cumulative survival of patients with CTD who underwent LT is similar to those with IPF and slightly less than those with COPD, with an increased risk of mortality that was most prominent at 6 months after transplant followed by subsequent narrowing of the survival differences over time. Lung transplantation may be a viable therapeutic option for patients with end-stage lung dysfunction resulting from a CTD.

Giant cell arteritis.

This review summarizes the diagnosis, clinical manifestations and management of giant cell arteritis. Giant cell arteritis is an immune-mediated vasculitis of medium to large sized arteries that affects individuals older than the age of 50. Patients typically present with signs of vascular insufficiency of the extracranial arteries of the head and systemic inflammation. Temporal artery biopsy remains the hallmark of diagnosis. Specific treatment regimens must be tailored to each individual, but steroids remain the backbone of therapy.

Sitagliptin-induced bilateral Achilles tendinitis

Sitagliptin (Januvia) is a widely used oral dipeptidyl peptidase-4 (DPP-4) inhibitor for the treatment of diabetes. Studies suggest that the drug is well-tolerated and no musculoskeletal side effects have been reported [1]. A 56-year-old female developed bilateral Achilles tendinitis 4 months after restarting sitagliptin. There was no trauma to the Achilles tendons, but she had recently started a low-impact exercise programme; she stopped exercising with no relief. She had no history of SpA, inflammatory arthritis or quinolone exposure and her HLA-B27 status was unknown. Physical therapy did not resolve her symptoms. She then tried a controlled ankle motion boot, ice application, AchilloTrain braces, ViscoHeel lifts and two additional courses of physical therapy over the ensuing 9 months without improvement. Bilateral ankle MRI confirmed insertional Achilles tendinosis on the left and low-grade insertional tendinitis of the right Achilles tendon (Fig. 1). Eventually her sitagliptin was discontinued because of the temporal relationship of her symptoms to restarting sitagliptin. After 4 weeks the patient reported complete resolution of her Achilles pain on the left and >50% improvement on the right. She has remained off sitagliptin and has had no further problems with tendinitis since that time.

Giant Cell Arteritis: Oral Versus Intravenous Corticosteroids

This review summarizes the current literature on the use of oral versus intravenous steroids for giant cell arteritis. Giant cell arteritis is an immune-mediated vasculitis of medium to large sized arteries that affects individuals older than the age of fifty. Patients typically present with signs of vascular insufficiency of the extracranial arteries of the head and systemic inflammation. Steroids remain the backbone of therapy, but the dose, maintenance and route of administration remain debatable.

Biologic Therapy for the Treatment of Giant Cell Arteritis

Abstract Giant cell arteritis (GCA), a vasculitis of the medium and large arteries, is traditionally managed with glucocorticoids. However, the side effects of chronic glucocorticoid use and the occurrence of refractory cases warrant the consideration of steroid-sparing agents, including biologic agents. Interleukin-6 (IL-6) inhibition shows the most promise as biologic therapy for refractory cases of GCA, but data to support the use of other existing biologic agents are currently lacking. A better understanding of the pathogenesis of GCA as well as clinical trials investigating both existing and emerging biologic agents is needed to expand therapeutic options for the treatment of GCA.

Review of Biologic Therapies

The emergence of biologic therapy as treatment for autoimmune and inflammatory diseases is rapidly changing the face of medicine. From fusion proteins to monoclonal antibodies, biologic therapies encompass a variety of agents that target distinct pathways involved in autoimmunity and inflammation. In this review, a survey of available and emerging biologic therapies detailing their current indications, mechanism of action, dosing, side effects, and recommended drug monitoring will be summarized so as to aid clinicians in making treatment decisions regarding their usage.

Pegylated interferon and ribavirin treatment in patients with hepatitis C virus associated vasculitis

Pegylated interferon and ribavirin treatment in patients with hepatitis C virus associated vasculitis