Heidi Roppelt

Prophylactic antibiotic usage for Pneumocystis jirovecii pneumonia in patients with systemic lupus erythematosus on cyclophosphamide: a survey of US rheumatologists and the review of literature.

Background:Use of cyclophosphamide in systemic lupus erythematosus (SLE) is associated with Pneumocystis jirovecii pneumonia (PJP) that has substantial morbidity and mortality. However, the frequency of PJP in these patients is unknown and there are no guidelines for prophylactic antibiotics. Objectives:The objectives of this study are to evaluate the frequency of PJP and the need for prophylactic antibiotics in these patients. Methods:We estimated incidence of PJP and use of prophylactic trimethoprim-sulfamethoxazole in these patients by a literature search and an e-mail survey of US rheumatologists. Results:We identified 18 manuscripts dealing with infections in SLE patients treated with cyclophosphamide. In these manuscripts, 121 cases of PJP were identified in 76,156 SLE patients with a frequency of 15.88 per 10,000 patients.Of 264 rheumatologists surveyed, 133 (50.37%) were using prophylactic antibiotics in these patients. One hundred thirty-one (49.63%) respondents did not use prophylactic antibiotics. 5,174 SLE patients received cyclophosphamide in last 5 years with 19.6 ± 30.6 (mean ± SD) patients per rheumatologist. 32 cases of PJP were reported. The total cumulative experience of 264 rheumatologists was 4742 years [(17.96 ± 10.35) (mean ± SD)] with a PJP rate of 67.48 per 10,000 years of practice. Conclusions:The frequency of PJP in SLE patients on cyclophosphamide remains low (0.1588%). Therefore, routine use of trimethoprim-sulfamethoxazole for PJP prophylaxis in SLE patients on cyclophosphamide does not appear to be substantiated by this study, except in those with elevated risk, ie, with severe leucopenia, lymphopenia, high dose corticosteroids, hypocomplementemia, active renal disease, and higher mean SLEDAI score. There is a need for consensus guidelines addressing prophylactic antibiotics in these patients.

Dramatic response to infliximab in refractory neurosarcoidosis

Sarcoidosis is a systemic disease characterized by noncaseating granulomas in the involved organs. Neurologic manifestations involving the central and/or peripheral nervous system occur in about 5% of patients. Neurosarcoidosis is often refractory to conventional treatment and therefore more effective treatment options are needed. While the etiology of the disease is still unknown, there is now a better understanding of its pathogenesis on a molecular level. It is clear that tumor necrosis factor-α (TNFα) plays a pivotal role in the development of the granulomas and it is believed to be a key cytokine involved in the pathogenesis of the disease. Taking advantage of this better understanding of disease pathogenesis, anti-TNFα agents are being increasingly used to treat refractory sarcoidosis. We report a patient with refractory neurosarcoidosis who showed dramatic improvement in the clinical and radiological manifestations following treatment with infliximab; he suffered a relapse upon discontinuation of the medication.

Successful remission of thrombotic thrombocytopenic purpura with rituximab in a patient with undifferentiated connective tissue disorder.

Thrombotic thrombocytopenic purpura (TTP) is rarely associated with undifferentiated connective tissue disorder. We present a patient with TTP, undifferentiated connective tissue disorder, and very high titer of anti-ribonucleoprotein antibodies. Her TTP did not respond to intravenous methylprednisolone and cyclophosphamide requiring her to remain dependent on plasmapheresis. Her disease remitted successfully after 4 doses of rituximab given at weekly intervals and she remained in continuous remission 6 months after therapy. We propose early and aggressive use of B-cell depletion therapy in TTP associated with autoimmune disorders.

Assessment of American College of Rheumatology-Endorsed Quality Indicators in Rheumatoid Arthritis Patients: A Quality Improvement Initiative.

BackgroundThe American College of Rheumatology endorses 7 rheumatoid arthritis (RA) quality indicators (QIs), which we used to access quality of care at our institution. ObjectiveThe aim of this study was to assess the quality of care provided to RA patients at our outpatient rheumatology practice based on adherence to 7 QIs. MethodsWe performed a retrospective paper chart review and included 356 RA patients to determine adherence to each QI. A &khgr;2 test analyzed trends in the assessment of disease activity and functional status. ResultsThere was excellent adherence to disease-modifying antirheumatic drug therapy (99.4%) and managing worsening disease (100%). Assessment of disease activity and functional status increased over the study period (72.8% to 94.2% and 70.8% to 93.4%, respectively). Despite this, none of our patients had disease prognosis classified and documented. Tuberculosis screening was done in 87.9%. Only a small percentage (1.4%) of patients met criteria for a glucocorticoid management plan, thus limiting our assessment of this QI. ConclusionsExcellent adherence to disease-modifying antirheumatic drug therapy and management is likely due to targeting clinical remission. Assessment of disease activity and functional status not only rose each year, but also is higher compared with similar studies. This may be due to an increased awareness of QIs and the utility of objective measures of disease activity. Deficient documentation of prognosis may be due to a lack of awareness of its importance. Suboptimal tuberculosis screening may be an artifact of poor documentation. We propose interventions to improve adherence.

High mercury seafood consumption associated with fatigue at specialty medical clinics on Long Island, NY.

We investigated the association between seafood consumption and symptoms related to potential mercury toxicity in patients presenting to specialty medical clinics at Stony Brook Medical Center on Long Island, New York. We surveyed 118 patients from April–August 2012 about their seafood consumption patterns, specifically how frequently they were eating each type of fish, to assess mercury exposure. We also asked about symptoms associated with mercury toxicity including depression, fatigue, balance difficulties, or tingling around the mouth. Of the 118 adults surveyed, 14 consumed high mercury seafood (tuna steak, marlin, swordfish, or shark) at least weekly. This group was more likely to suffer from fatigue than other patients (p = 0.02). Logistic regression confirmed this association of fatigue with frequent high mercury fish consumption in both unadjusted analysis (OR = 5.53; 95% CI: 1.40–21.90) and analysis adjusted for age, race, sex, income, and clinic type (OR = 7.89; 95% CI: 1.63–38.15). No associations were observed between fish intake and depression, balance difficulties, or tingling around the mouth. Findings suggest that fatigue may be associated with eating high mercury fish but sample size is small. Larger studies are needed to determine whether fish intake patterns or blood mercury tests warrant consideration as part of the clinical work-up in coastal regions.

Intestinal Infarction and Portal Vein Thrombosis in a Patient with Henoch Schonlein Purpura

Henoch Schonlein purpura is a systemic vasculitis that commonly affects children and teenagers but also affects adults of all ages. In most instances it has a benign course. Organ involvement, particularly in adults, and notably the kidneys and gastrointestinal tract may require therapeutic intervention and may have a less favorable outcome. We report a case of a 58-year-old man who presented with purpura and who rapidly developed catastrophic intestinal vasculitis, leading to his demise.