Bella Mehta

Life-threatening complications of adult-onset Still's disease.

Adult-onset Still’s Disease (AOSD) since its description in 1971 has proven to be a very complex and challenging disease entity. This rare auto-inflammatory disease is classically described by the “Still’s triad” of fever, rash, and arthritis, although the atypical cases frequently outnumber the typical ones. The exact pathogenesis and etiologic factors responsible for the clinical features remain largely obscure, despite recent suggestive cytokine biology findings. Diagnosis is made on clinical grounds, following the exclusion of mimickers of infectious, autoimmune or neoplastic etiology, with the additional consideration of non-specific laboratory abnormalities such as peripheral leukocytosis and elevation of serum ferritin and other acute phase reactants. The disease manifestations are protean and can include diverse complications, affecting multiple organ systems. Moreover, the severity of the organ involvement can vary considerably, representing a wide spectrum from the self-limited to severe. The mainstay of therapy has evolved from the traditional use of corticosteroids and oral immunosupressants to the newer targeted treatments with biologic agents. The scope of this review is to alert the clinician to the existence of life-threatening AOSD complications, namely the macrophage activation syndrome, disseminated intravascular coagulopathy, thrombotic thrombocytopenic purpura, diffuse alveolar hemorrhage, and pulmonary arterial hypertension. Such knowledge may lead in earlier recognition, prompt treatment, and, ideally, improved patient outcomes.

Erratum to “Ferritin in Adult-Onset Still’s Disease: Just a Useful Innocent Bystander?”

There is an error in the citations of our previous paper as reference [51] G. Zandman-Goddard, M. Blank, P. Langevitz et al., “Antiserum amyloid component P antibodies in patients with systemic lupus erythematosus correlate with disease activity,” Annals of the Rheumatic Diseases, vol. 64, no. 12, pp. 1698–1702, 2005 should be replaced by the following references: [1] Li, X., et al., “Epidermal Growth Factor-Ferritin H-Chain Protein Nanoparticles for Tumor Active Targeting,” Small, 2012; [2] Jezequel, P., et al., “Validation of tumor-associated macrophage ferritin light chain as a prognostic biomarker in node-negative breast cancer tumors: A multicentric 2004 national PHRC study,” International Journal of Cancer, 2012. 131(2): p. 426–37. [3] Pitcher, J., et al., “Disruption of neuronal CXCR4 function by opioids: preliminary evidence of ferritin heavy chain as a potential etiological agent in neuroAIDS,” Journal of Neuroimmunology, 2010. 224(1-2): p. 66–71. [4] Rosen, H. R., “Placental isoferritin-associated p43 in pregnancy and breast cancer: minireview,” Neoplasma, 1996. 43(6): p. 357–62.

Ferritin in Adult-Onset Still's Disease: Just a Useful Innocent Bystander?

Background. Adult-Onset Stills Disease (AOSD) is an immune-mediated systemic disease with quotidian-spiking fever, rash, and inflammatory arthritis. Hyperferritinemia is a prominent feature, often used for screening. Methods. The key terms “ferritin” and “hyperferritinemia” were used to search PubMed and Medline and were cross-referenced with “Stills Disease.” Results. Hyperferritinemia, although nonspecific, is particularly prevalent in AOSD. While most clinicians associate ferritin with iron metabolism, this is mostly true for the H isoform and not for the L isoform that tends to increase dramatically in hyperferritenemia. In these situations, hyperferritinemia is not associated with iron metabolism and may even mask an underlying iron deficiency. We review, in systematic fashion, the current basic science and clinical literature regarding the regulation of ferritin and its use in the diagnosis and management of AOSD. Conclusion. Serum hyperferritinemia in AOSD has been described for 2 decades, although its mechanism has not yet been completely elucidated. Regulation by proinflammatory cytokines such as interleukin (IL)-1b, IL-6, IL-18, MCSF, and INF-α provides a link to the disease pathogenesis and may explain rapid resolution of hyperferritinemia after targeted treatment and inhibition of key cytokines.

Screening Optimization of Latent Tuberculosis Infection in Rheumatoid Arthritis Patients

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.

CEPP regimen (cyclophosphamide, etoposide, procarbazine and prednisone) as initial treatment for Hodgkin lymphoma patients presenting with severe abnormal liver function

ABVD regimen (doxorubicin, bleomycin, vinblastine and dacarbazine) remains the most commonly used front-line therapy for Hodgkin lymphoma. However, atypical and extranodal presentations present challenges to initial therapy, especially in the presence of renal and liver failure. We hereby present two cases of young male patients with atypical presentation of Hodgkin lymphoma with severe abnormal liver function. Patients showed excellent response to cyclophosphamide, etoposide, procarbazine and prednisone (CEPP regimen).

Disparities in Outcomes for Blacks versus Whites Undergoing Total Hip Arthroplasty: A Systematic Literature Review

Objective. Total hip replacement (THA) surgery is a successful procedure, yet blacks in the United States undergo THA less often and reflect poorer outcomes than whites. The purpose of this study is to systematically review the literature on health-related quality of life after THA, comparing blacks and whites. Methods. A librarian-assisted search was performed in Medline through PubMed, Embase, and Cochrane Library on February 27, 2017. Original cohort studies examining pain, function, and satisfaction in blacks and whites 1 year after elective THA were included. Using the Patient/Population–Intervention–Comparison/Comparator–Outcome (PICO) process format, our population of interest was US black adults, our intervention was elective THA, our comparator was white adults, and our outcomes of interest were pain, function, and satisfaction after elective THA. The protocol was registered under the PROSPERO international register, and the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines were followed. Results. Of the articles, 4739 were screened by title, 180 by abstract, 25 by full text, and 4 remained for analysis. The studies represented 1588 THA patients, of whom 240 (15%) were black. All studies noted more pain and worse function for blacks; although differences were statistically significant, they were not clinically significant. One study sought and identified less satisfaction for blacks after THA, and 1 study showed worse fear and anxiety scores in blacks. Conclusion. When measured, there are small differences in THA outcomes between blacks and whites, but most studies do not analyze/collect race. Future studies should address the effect of race and socioeconomic factors on healthcare disparities.

Education mitigates the effect of poverty on total knee arthroplasty outcomes

Total knee arthroplasty (TKA) outcomes are worse for patients from poor neighborhoods, but whether education mitigates the effect of poverty is not known. We assessed the interaction between education and poverty on 2‐year Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) pain and function.

Cardiac papillary fibroelastoma: The need for a timely diagnosis

Cardiac papillary fibroelastomas (CPFs) are the second most common primary cardiac tumors and the most common cardiac valvular tumors. Although they are histologically benign and usually asymptomatic, CPFs can lead to serious and life-threatening complications like myocardial infarction, stroke, pulmonary embolus, cardiac arrest etc. CPFs represent a rare entity in clinical medicine and literature regarding their management is limited. We report two cases which illustrate such complications arising from undiagnosed CPFs on the aortic valve. We further stress on the importance of identifying CPFs early so that they can be managed appropriately based on recommendations from the available literature.

Radiographic Improvement in Sarcoid Arthropathy after Infliximab Treatment

To the Editor: We describe our experience on the use of infliximab in sarcoid arthropathy and partial reversal of the typical radiographic changes seen in sarcoid arthropathy after treatment. A 35-year-old African American man presented 5 years ago with bilateral hand arthritis, dactylitis, and a shiny, fleshy growth over the tip of the nose. Examination revealed bilateral proximal interphalangeal joint synovitis and severe deformity and resorption of the distal interphalangeal joints with dystrophic nail changes and diffuse dactylitis. The fleshy growth was indicative of a sarcoid-specific “lupus pernio” presentation, confirmed by skin biopsy, showing noncaseating granulomas. Chest radiography showed paratracheal and bilateral hilar lymphadenopathy without parenchymal disease. Laboratory evaluation revealed elevation of … Address correspondence to Dr. P. Efthimiou, Rheumatology Division, Lincoln Medical and Mental Health Center, 234 East 149th St., New York, NY 10451, USA. E-mail: pe53{at}cornell.edu

Adult-Onset Still’s Disease and Macrophage-Activating Syndrome Progressing to Lymphoma

A 66-year-old man with adult-onset Still’s disease (AOSD) and macrophage-activation syndrome (MAS) presented with four weeks of progressive fatigue and weakness. Past history was notable for gout and a deep vein thrombosis provoked after a long flight, and there was no family history of autoimmune conditions or malignancy. Two and a half years prior, he developed anemia, thrombocytopenia, hypoalbuminemia, and hyperferritinemia, followed by fatigue, weight loss, fever, and night sweats. He had no rash, arthritis, arthralgias, or pleuritic chest pain. On physical examination, he had splenomegaly with mild lymphadenopathy. The patient underwent an extensive workup including malignancy screening, in which cross-sectional imaging confirmed splenomegaly and scattered lymphadenopathy. Bone marrow biopsy was hypercellular, with normal cell lines and no hemophagocytosis. Left inguinal lymph node biopsy showed reactive hyperplasia, suggestive of Castleman’s disease-like features (Fig. 1a). Several months later he developed arthralgias, mild arthritis, and rash, along with continued fever, fatigue, cytopenia, and hyperferritinemia. A diagnosis of MAS in the setting of AOSD was made. He was treated with methylprednisolone 100 mg daily and anakinra 100 mg daily with rapid resolution of symptoms. Over the next six months, steroids and anakinra were tapered off completely. Three months after discontinuation of anakinra and one month prior to his in-hospital presentation, the patient developed recurrent fatigue and weight loss, without fever, rash, or localizing symptoms. Physical examination was again notable for lymphadenopathy; laboratory tests revealed recurrent anemia, thrombocytopenia, hypoalbuminemia, and hyperferritinemia. He was restarted on anakinra and prednisone 100 mg daily for treatment of presumed relapse of MAS/ AOSD but without improvement. The patient was rehospitalized for further evaluation and management. He was noted to have diffuse lymphadenopathy and hepatosplenomegaly on cross-sectional imaging, and interleukin (IL)-6 and tumor necrosis factor (TNF)-α levels were elevated. Laboratory findings were notable for leukopenia, profound anemia and thrombocytopenia, hypoalbuminemia, HSSJ (2018) 14:214–221 DOI 10.1007/s11420-018-9606-8