Carmen Gota

Anti-tumour necrosis factor therapy in patients with refractory Takayasu arteritis: long-term follow-up

Objective: To assess the efficacy of anti-tumour necrosis factor (TNF) therapy to induce remission in patients with Takayasu arteritis (TAK) refractory to other immunosuppressive therapies. Methods: Retrospective single-centre study of 25 patients with refractory TAK. Results: Patients were treated with infliximab (IFX) or etanercept (ETA) for up to 7 years; 21 with IFX (median 28 months (range 2–84)) and 9 with ETA (median 28 months (range 4–82)); 5 patients initially treated with ETA subsequently switched to IFX. Following anti-TNF therapy, remission was achieved and prednisone was discontinued in 15 patients (60%) and successfully tapered below 10 mg/day in an additional 7 patients (28%). Of 18 patients treated with other immunosuppressive agents concurrent with anti-TNF therapy, 9 (50%) could taper or discontinue the additional agent. Major relapses occurred in four patients that initially achieved stable remission. Four patients suffered adverse events, including one with opportunistic infections and one with breast cancer. Conclusions: In this group of patients with refractory TAK, anti-TNF therapy was associated with remission in a majority of patients, facilitating dose reduction or discontinuation of prednisone and other immunosuppressive therapy. These findings strengthen the rationale for the conducting of a randomised controlled trial of anti-TNF therapy in TAK.

Induction of Clinical Autoimmune Disease by Therapeutic Interferon-α

Much progress has been made in our understanding of Interferon biology since its initial description by Isaacs and Lindenmann in 1957. Type I Interferons have well established direct antiviral, antiproliferative and immunomodulatory effects. IFN-a has been used with various degrees of success in the treatment of viral, autoimmune (AI) and malignant diseases such as: chronic viral hepatitis B and C, Behcet’s disease, cryoglobulinemia, chronic myelogenous leukemia (CML), malignant non Hodgkin lymphoma, multiple myeloma (MM), hairy cell leukemia (HCL), AIDS related Kaposi sarcoma, condylomata acuminata and carcinoid syndrome, to mention just a few.

Lupus-related hepatitis : Complication of lupus or autoimmune association? case report and review of the literature

Patients with systemic lupus erythematosus (SLE) have a25–50%chanceofdevelopingabnormallivertestsintheirlifetimes (1, 2). Prior treatment with potentially hepato-toxic drugs or viral hepatitis has usually been implicatedas the main cause of liver disease in SLE patients. Aftercareful exclusion of these etiologies, the physician maybe confronted with the dilemma of whether to classify thepatient as having a primary liver disease with associatedautoimmune clinical and laboratory features resemblingSLE, such as autoimmune hepatitis (AIH), or as havingliver disease as a manifestation of SLE. We present a caseof hepatitis occurring in an SLE patient in which no viralor drug-related etiology could be found. We review bothentitiesandprovideasimplifiedguidetohelpdifferentiateSLE-related hepatitis from AIH.

Fibromyalgia and bipolar disorder: a potential problem?

OBJECTIVE To screen patients with fibromyalgia for bipolar disorder and to determine if there were any clinical clues, other than the Mood Disorders Questionnaire (MDQ), which might suggest a diagnosis of comorbid bipolar disorder. METHODS A total of 128 consecutive new fibromyalgia patients referred to a tertiary care center rheumatology practice were enrolled and assessed using a standard clinical protocol that included the completion of four screening questionnaires: (i) MDQ for bipolar disorder, (ii) Beck Depression Inventory (BDI) for depression, (iii) Epworth Sleepiness Scale (ESS) for daytime sleepiness, and (iv) Fibromyalgia Impact Questionnaire Disability Index (FIQ-DI) to assess for functional capacity. RESULTS A quarter of the fibromyalgia subjects, 25.19%, had a positive screen for bipolar disorder (MDQ >or= 7); 78.12% were clinically depressed (BDI >or= 10); and 52.13% reported daytime sleepiness (ESS >or= 10). Fibromyalgia subjects who screened positive for bipolar disorder had more severe depression than those with a negative screen [median BDI: 26.0 (19.0, 32.0) versus 15.0 (9.0, 24.0), p < 0.001]. CONCLUSIONS We report a high prevalence of positive testing for bipolar disorder in this fibromyalgia cohort. Clinical data and questionnaire instruments other than nonspecific high depression severity failed to identify these patients. Since the norepinephrine serotonin reuptake inhibitors duloxetine and milnacipran have been recently approved by the U.S. Food and Drug Administration for the treatment of fibromyalgia, and because patients with bipolar disorder may experience destabilization of mood when treated with such agents, patients with fibromyalgia should be systematically screened for bipolar disorder prior to treatment.

Fibromyalgia and Obesity: The Association Between Body Mass Index and Disability, Depression, History of Abuse, Medications, and Comorbidities.

AimThe aim of this study was to determine the frequency of increasing body mass index (BMI) in fibromyalgia (FM) and to understand the impact of increasing BMI on FM. MethodsPatients with FM were divided into 3 BMI classifications: normal weight, overweight, and obese. We then sought relationships of increasing BMI to core process FM variables and symptoms and disability, as well as medical comorbidities and demographic, socioeconomic, psychiatric, and treatment data. ResultsOf 224 patients, 0.4% were underweight; 25.9%, normal weight; 29.9%, overweight; 43.8%, obese. We found no differences within groups with regard to age, gender, demographics, FM symptoms, FM impact questionnaire scores, and meeting the American College of Rheumatology 1990 criteria and FM survey criteria. Patients with FM who are obese, compared with normal-weight patients, have higher depression scores measured by Patient Health Questionnaire 9 (13.2 [6.6] vs 10.5 [6], P = 0.03), report increased disability by Health Assessment Questionnaire Disability Index scores (1.3 [0.6] vs 0.9 [0.6], P < 0.001), exercise less (8.4% vs 25.4%, P = 0.003), have more medical comorbidities (1.5 [1.3] vs 0.7 [0.9], P < 0.001), take more medications for FM (3.5 [2.2] vs 2.1 [1.8], P < 0.001), and report higher prevalence of abuse (48% vs 33.9%, P = 0.016) and sexual abuse (17.3% vs 6.8%, P = 0.01). ConclusionsCompared with normal-weight patients, obese FM patients are more disabled, report more medical comorbidities, exercise less, have a higher incidence of abuse, report increased depressive symptoms, and take more medications for FM. Bivariate analysis showed association of increasing BMI with the Health Assessment Questionnaire Disability Index (not FM impact questionnaire) and depression. We confirm that the prevalence of overweight and obesity is high in FM and believe that physicians treating FM should be aware of our bivariate linear correlations and discuss weight loss with their FM patients. Even if increasing BMI is not intrinsic to FM, it contributes to poor mood and functional outcome and should be a treatment goal.

Clinical significance of immunoglobulin deposition in leukocytoclastic vasculitis: a 5-year retrospective study of 88 patients at cleveland clinic.

Objective:To study the diagnostic utility and clinical associations of immunoglobulin deposition, determined by direct immunofluorescence (DIF) in cutaneous leukocytoclastic vasculitis (LCV). Methods:We performed a retrospective study of all biopsy-proven LCV cases seen at Cleveland Clinic between 2007 and 2012. All LCV cases in which DIF was performed were included. Results:Of the 218 LCV cases, 106 cases had DIF performed and data from 88 cases were available: median (SD) age 53.3 (19.4), 52% male, 64.1% white, duration of rash 5.5 (20.8) months; follow-up 14 (19.7) months. DIF results showed any immunoglobulin and/or complement and/or fibrinogen in 70.5%, immunoglobulin A (IgA) in 36.4%, immunoglobulin M (IgM) in 21.6%, immunoglobulin G (IgG) in 11.4%. Patients with IgA deposition by DIF, compared with those without IgA, were younger, 44 (19) versus 56 (17) (P = 0.006), more likely to be white (P = 0.025) and had more organs affected by vasculitis (P = 0.002), higher incidence of gastrointestinal tract involvement (P = 0.0001) and renal disease (P = 0.006). No differences between rates of infection or malignancy were seen between DIF IgA, IgM, or IgG–positive versus negative patients. Conclusions:In patients with cutaneous LCV, IgA is the most common immunoglobulin found by DIF. IgA deposition, but not IgM or IgG, is predictive of associated renal and gastrointestinal organ involvement by vasculitis. No association between the type of immunoglobulin and preexisting infection or malignancy was found. DIF results add information that is clinically relevant to the diagnosis and management of LCV.

The impact of depressive and bipolar symptoms on socioeconomic status, core symptoms, function and severity of fibromyalgia.

To evaluate the prevalence of depressive and bipolar symptoms in a cohort of consecutive fibromyalgia (FM) patients seen in a tertiary care center and to determine the relationship between depressive and manic symptoms with FM symptoms, socioeconomic status, severity and function.

Serum chitotriosidase activity and Wegener's granulomatosis

OBJECTIVE We evaluated serum chitotriosidase activity in patients with Wegeners granulomatosis (WG) and compared the values to controls. DESIGN AND METHODS We used a standard fluorometric assay to measure chitotriosidase enzyme activity. RESULTS Serum chitotriosidase enzyme activity levels were higher in WG patients. We found no association between clinical disease activity and chitotriosidase enzyme activity. CONCLUSIONS Serum chitotriosidase enzyme activity has limited utility as a biomarker in WG patients.

Inflammatory abdominal aortic aneurysm with retroperitoneal fibrosis.

A 69-year-old woman was transferred to our intensive care unit with concerns of a possible rupture of abdominal aortic aneurysm (AAA). She presented with a 1-week history of sharp left upper quadrant abdominal pain radiating to her back. Her comorbidities included hypertension and chronic obstructive pulmonary disease with a >90 pack-year history of smoking. On examination, she was hemodynamically stable but was cachectic in appearance and had epigastric tenderness. Laboratory evaluation revealed hemoglobin of 11g/dL, white cell count of 12 900 k/uL, and creatinine of 0.4 mg/dL. Both the erythrocyte sedimentation rate and c-reactive protein were elevated (100 mm/h and 6.9 mg/dL, respectively). Computed tomography scan performed at the outside facility demonstrated diffuse aortic plaque with descending thoracic aortic aneurysm measuring 4.2 cm (Figure 1, arrow) and a …

Malignancy-associated dermatomyositis: Retrospective case–control study from a single tertiary care center

Comprehensive Cancer Center and Connecticut Challenge, National Cancer Institute of the National Institutes of Health (F32 CA144335), Mentored Research Scholar Grant in Applied and Clinical Research, from the American Cancer Society (MRSG-13-016-01-CPPB), and Yale SPORE in Skin Cancer funded by the National Cancer Institute of the National Institutes of Health (P50 CA121974 [R. Halaban, principal investigator]).