Dermatomyositis presenting with diffuse calcinosis.

We present the case of a woman in her early 50s who initially presented to an orthopedist for nodules located near the posterior knee. Imaging revealed diffuse subcutaneous calcifications and she was subsequently referred to rheumatology. Additional testing included myositis panel, electromyography (EMG) and muscle biopsy which indicated the presence of an inflammatory myopathy. It was determined that this patient had an uncommon presentation of dermatomyositis in which her primary complaint was calcinosis cutis. While rash and muscle weakness are often the symptoms most commonly associated with dermatomyositis, it is essential to have a wide differential for patients presenting with calcium deposition in soft tissues. This is particularly important in patients with certain antibodies, including the NXP-2 antibody, which can be associated with malignancy and should prompt an appropriate malignancy workup.

Multidisciplinary diagnosis and management of inflammatory aortic aneurysms.

BACKGROUND: Inflammatory abdominal aortic aneurysms (IAAAs) are a variant involving a distinct immunoinflammatory process, with nearly one half believed to be associated with IgG4-related disease (IgG4-RD). METHODS: MEDLINE and Google Scholar searches were conducted for English-language publications relevant to inflammatory aortic aneurysms from January 1970 onward. The search terms included inflammatory aortic aneurysms, aortitis, periaortitis, IgG4-related disease, and retroperitoneal fibrosis. Relevant studies were selected for review based on their relevance. RESULTS: Morphologically, IAAAs are characterized by a thickened aneurysm wall often displaying contrast enhancement and elevated metabolic activity on fluorine-18 fluorodeoxyglucose-positron emission tomography imaging. A strong association exists with perianeurysmal and retroperitoneal fibrosis. Although the rupture risk appears lower with IAAAs than with noninflammatory abdominal aortic aneurysms (AAAs), the currently recommended diameter threshold for operative management is the same. Open repair has been associated with increased morbidity compared with noninflammatory AAAs, and a retroperitoneal approach or minimal dissection transperitoneal approach has been recommended to avoid duodenal and retroperitoneal structural injuries. Endovascular aneurysm repair has been increasingly used, especially for patients unfit for open surgery. It is important to exclude an infectious etiology before the initiation of immunosuppressive therapy or operative repair. Multimodality imaging follow-up is critical to monitor disease activity and secondary involvement of retroperitoneal structures by the associated fibrotic process. Maintenance of immunosuppressive therapy will be needed postoperatively for most patients with active systemic disease, especially those with IgG4-RD and those with persistent symptoms. Additional interventions aimed at ureteral decompression could also be required, and lifelong follow-up is mandatory. CONCLUSIONS: Preoperative multimodality imaging is a diagnostic cornerstone for assessment of the disease extent and activity. IgG4-RD is an increasingly recognized category of IAAAs, with implications for tailoring adjunctive medical therapy. Open surgical repair remains the procedure of choice, although endovascular aneurysm repair is increasingly being offered. Maintenance immunosuppressive therapy can be offered according to the disease activity as assessed by follow-up imaging studies.

Patient Perspectives on Smoking Cessation and Interventions in Rheumatology Clinics.

OBJECTIVE: Although smoking is a risk factor for cardiovascular and rheumatic disease severity, only 10% of rheumatology visits document cessation counseling. After implementing a rheumatology clinic protocol that increased tobacco quitline referrals 20-fold, we undertook this study to examine patients' barriers and facilitators to smoking cessation based on prior rheumatology experiences, to solicit reactions to the new cessation protocol, and to identify patient-centered outcomes or signs of cessation progress following improved care. METHODS: We recruited 19 patients who smoke (12 with rheumatoid arthritis [RA] and 7 with systemic lupus erythematosus [SLE]) to participate in 1 of 3 semistructured focus groups. Transcripts of the focus group discussions were analyzed using thematic analysis to classify barriers, facilitators, and signs of cessation progress. RESULTS: Participant-reported barriers and facilitators to cessation involved psychological, health-related, and social and economic factors, as well as health care messaging and resources. Commonly discussed barriers included viewing smoking as a crutch amid rheumatic disease, rarely receiving cessation counseling in rheumatology clinics, and very limited awareness that smoking can worsen rheumatic diseases or reduce efficacy of some rheumatic disease medications. Participants endorsed our cessation protocol with rheumatology-specific education and accessible resources, such as a quitline. Beyond quitting, participants prioritized knowing why and how to quit as signs of progress outcomes. CONCLUSION: Focus groups identified themes and categories of facilitators/barriers to smoking cessation at the levels of patient and health system. Two key outcomes of improving cessation care for patients with RA and SLE were knowing why and how to quit. Emphasizing rheumatologic health benefits and cessation resources is essential when designing and evaluating rheumatology smoking cessation interventions.

A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome.

BACKGROUND: Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy (IgM in more than 90% of the cases). It is difficult to distinguish from other neutrophilic urticarial dermatoses, and diagnosis is based on the Strasbourg criteria. Interleukin-1 is considered the key mediator, and interleukin-1 inhibitors are considered first line treatment. Here, we present two cases of Schnitzler syndrome, both successfully treated with anakinra. OBJECTIVES: To increase awareness regarding clinical presentation, diagnosis, and treatment of this rare disorder. CASES: We describe the clinical features and disease course of two patients with Schnitzler syndrome, diagnosed using the Strasbourg criteria. Both were treated with anakinra with remarkable response to therapy. CONCLUSION: Schnitzler syndrome is a rare and underdiagnosed disorder. High suspicion should be maintained in patients with chronic urticaria-like dermatoses, intermittent fevers, and arthralgias. A serum protein electrophoresis and immunofixation should be performed in these patients. The diagnosis is important to recognize as Schnitzler syndrome is associated with malignancy. A lymphoproliferative disorder develops in about 20% of patients at an average of 7.6 years after onset of symptoms. Thus, patients warrant long-term follow-up. IL-1 inhibitors are extremely effective in relieving symptoms and are considered first line therapy.

Acute Central Vision Loss in an IV Drug User.

This report describes the case of a 21-year-old heroin user who presented with a 6-day history of decreased vision in her right eye, preceded by 1 week of headache and tender scalp nodules, neck stiffness, and photophobia. A broad infectious workup for acute vision loss was completed, and she was ultimately presumed to have acquired toxoplasmic chorioretinitis (ocular toxoplasmosis). We review the initial workup for chorioretinitis, and the epidemiology, diagnosis, and treatment of ocular toxoplasmosis. Intravenous drug users may be at increased risk of acquired ocular toxoplasmosis.