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This cohort study investigated the association between treatment of dermatomyositis with intravenous immunoglobulin and thromboembolic events.

Objectives To determine if serologic phenotypes could be identified in systemic lupus erythematosus patients developing interstitial lung disease (ILD) and/or myositis. Methods Adult SLE patients (without myositis/ILD at baseline) had annual assessments and serum sampling between 2000 and 2017. New-onset ILD was identified using the SDI pulmonary fibrosis item. New-onset myositis was identified using the SLICC Damage Index muscle atrophy/weakness item, the SLEDAI-2K item for myositis, and annual creatinine kinase testing. Chart review confirmed ILD/myositis cases and randomly sampled SLE patients from baseline formed our sub-cohort (N = 72). Cases and sub-cohort were compared regarding myositis-related biomarkers at baseline and at a randomly selected follow-up between baseline and end of observation (date of ILD/myositis diagnosis or Dec. 31, 2017). Descriptive analyses and hazards ratios (HRs) were generated for ILD/myositis incidence, focusing on baseline serology and adjusting for sex, race/ethnicity, age at SLE diagnosis, and SLE duration. Results Fourteen SLE patients developed ILD (N = 9), myositis (N = 3), and/or both (N = 2). Thirteen of those (92.9%) developing ILD/myositis had at least one biomarker at baseline, versus 47 (65.3%) SLE patients who never developed myositis/ILD. The most common biomarkers in myositis/ILD were KL-6, anti-Ro52, and anti-Ku. Baseline biomarkers tended to remain positive in follow-up. In multivariate Cox regressions, SLE patients had higher risk of developing myositis/ILD with elevated baseline KL-6 (adjusted hazard ratio 3.66; 95% confidence interval 1.01, 13.3). When updating biomarkers over time, we also saw correlations between anti-Smith and ILD/myositis. Conclusions Baseline myositis-related biomarkers were highly associated with ILD/myositis incidence. This is the first identification of biomarker phenotypes with ILD/myositis risk in SLE.

A 56-year-old woman presented with a 5-day history of arthralgias and an erythematous papular eruption affecting the face, arms, chest, and upper back, for which she was started on high potency topical corticosteroids. She was noted to have associated synovitis of the hands and wrists bilaterally and was started on celecoxib. One week later, the patient returned with worsening pain and swelling in her hands in addition to new confluent, somewhat firm, erythematous papulonodules on the face, trunk, forearms, and dorsal hands. Two biopsies were performed with evidence of dermal histiocytic proliferation consistent with multicentric reticulohistiocytosis (MRH). Mycobacterial and malignancy screening were completed, and the patient was started on hydroxychloroquine, methotrexate, and systemic corticosteroids. MRH is a rare histiocytic disorder predominantly affecting Caucasian women in the fourth decade of life. Associations include arthritis, which can be mutilating in nature, and malignancy. Given the frequently destructive nature of the associated arthritis, timely diagnosis and treatment is crucial and may prevent long-term disability. Teaching points: (I) highlight the clinical manifestations of MRH, including the differential diagnosis of arthritis-dermatitis syndromes. (II) Underscore that MRH can mimic dermatomyositis. (III) Describe the differential diagnosis of arthritis mutilans (e.g., RA, PsA, gout, MRH). (IV) Examine the evidence for therapeutic options in MRH.

Objectives Myositis is an infrequent feature of SLE and may often be overlooked. We aimed to estimate the incidence of myositis in SLE, and to determine demographic and clinical factors associated with it. Methods Within our lupus cohort, we identified potential myositis cases using the SLICC Damage Index for muscle atrophy or weakness, the SLEDAI-2K item for myositis, and annually measured serum creatinine kinase. Cases were confirmed through chart review. We performed descriptive analyses of prevalent myositis cases as of January 2000. From that point onward, we studies patients without myositis to determine risk of incident myositis, using cohort analyses adjusted for demographic variables (age, sex, race/ethnicity). Results As of January 2000, there were 5 prevalent myositis cases in our SLE cohort. Among 560 SLE patients with a study visit from January 2000 onward, with no history of myositis at baseline, 5 new cases (4 females, 1 male) were identified over an average follow-up of 8.5 years (incidence 1.05 cases per 1000 person-years). There was a higher proportion of Caucasians in the non-myositis group versus myositis group, with a trend for fewer females in the myositis cases. Arthritis, Raynaud’s phenomenon, and anti-Smith antibodies were common pre-existing features, occurring in all incident myositis cases. In Cox regression analyses adjusting for age, race/ethnicity and sex, non-Caucasian patients had a markedly increased risk of developing myositis. Conclusion We found a low incidence of myositis in our SLE cohort. A cluster of variables, particularly non-Caucasian race/ethnicity, arthritis, Raynaud’s phenomenon, and anti-Smith antibodies were associated with risk of developing myositis in SLE. These variables may aid clinicians in identifying SLE patients at highest risk for this important complication.

Dermatologists treating immune-mediated skin disease must now contend with the uncertainties associated with immunosuppressive use in the context of the SARS-CoV-2 pandemic. Though the risk of infection with many commonly used immunosuppressive agents remains low, direct data evaluating the safety of such agents in COVID-19 are scarce. This article reviews and offers guidance based upon currently available safety data, and the most recent COVID-19 outcome data, in patients with immune-mediated dermatologic disease. The interdisciplinary panel of experts emphasize a stepwise, shared decision-making approach in the management of immunosuppressive therapy. The goal of this article is to help providers minimize the risk of disease flares while simultaneously minimizing the risk of iatrogenic harm during an evolving pandemic., This article adds to the limited literature on COVID-19 and immunosuppression. It provides expert opinion based on existing drug safety data and recent COVID-19 outcome data in patients with immune-mediated dermatologic disorders. • The goal is to facilitate management of immunosuppressive drugs and minimize potential for harm in this patient population.

Purpose of review Sexual function and fertility are neglected topics in men with systemic lupus erythematosus (SLE) in the literature. This review examines the impact of SLE and its treatment on fertility and erectile function. Recent findings Systemic illness, such as chronic kidney disease, and drugs used in SLE are associated with male infertility and sexual dysfunction through changes at the level of the hypothalamic-pituitary axis and direct testicular damage. In SLE patients, evidence shows a dose-dependent gonadotoxicity associated with intravenous (IV) cyclophosphamide (CYC). In contrast, recent observational studies evaluating disease-modifying anti-rheumatic drugs, non-steroidal anti-inflammatory drugs, and corticosteroids have found little evidence suggesting a significant impact of paternal exposure on fertility or pregnancy outcomes. In most patients, infertility management is focused on controlling SLE disease activity, minimizing the dose of gonadotoxic medications and cryopreserving sperm prior to treatment with IV CYC. Summary Reproductive issues are not uncommon in males with SLE. Understanding the impact of disease activity and drug effects on reproductive health may avert irreversible infertility and improve patient quality of life. However, additional studies are required to further explore the impact of SLE and its treatment on male fertility.

Objective.To determine cancer incidence in a large pediatric-onset systemic lupus erythematosus (SLE) population.Methods.Data were examined from 12 pediatric SLE registries in North America. Patients were linked to their regional cancer registries to detect cancers observed after cohort entry, defined as date first seen in the clinic. The expected number of malignancies was obtained by multiplying the person-years in the cohort (defined from cohort entry to end of followup) by the geographically matched age-, sex-, and calendar year–specific cancer rates. The standardized incidence ratio (SIR; ratio of cancers observed to expected) was generated, with 95% CI.Results.A total of 1168 patients were identified from the registries. The mean age at cohort entry was 13 years (SD 3.3), and 83.7% of the subjects were female. The mean duration of followup was 7.6 years, resulting in a total observation period of 8839 years spanning the calendar period 1974–2009. During followup, fourteen invasive cancers occurred (1.6 cancers per 1000 person-yrs, SIR 4.13, 95% CI 2.26–6.93). Three of these were hematologic (all lymphomas), resulting in an SIR for hematologic cancers of 4.68 (95% CI 0.96–13.67). SIR were increased for both male and female patients, and across age groups.Conclusion.Although cancer remains a relatively rare outcome in pediatric-onset SLE, our data do suggest an increase in cancer for patients followed an average of 7.6 years. About one-fifth of the cancers were hematologic. Longer followup, and study of drug effects and disease activity, is warranted.

Objective.Pain is one reason some rheumatology patients may consider use of medical cannabis, a product increasingly perceived as a safe and neglected natural treatment option for many conditions. Legalization of recreational cannabis in Canada will promote access to cannabis. Physicians must therefore provide patients with the best evidence-based information regarding the medicinal effects and harm of cannabis.Methods.The Canadian Rheumatology Association (CRA) mandated the development of a position statement for medical cannabis and the rheumatology patient. The current literature regarding the effects of medical cannabis for rheumatology patients was assessed, and a pragmatic position statement to facilitate patient care was developed by the Therapeutics Committee of the CRA and approved by the CRA board.Results.There are no clinical trials of medical cannabis in rheumatology patients. Evidence is insufficient about the benefit of pharmaceutical cannabinoids in fibromyalgia, osteoarthritis, rheumatoid arthritis, and back pain, but there is evidence of a high risk of harm. Extrapolating from other conditions, medical cannabis may provide some symptom relief for some patients. Short-term risks of psychomotor effects can be anticipated, but longterm risks have not been determined and are of concern.Conclusion.Despite lack of evidence for use of medical cannabis in rheumatology patients, we acknowledge the need to provide empathetic and pragmatic guidance for patient care. This position statement aims to facilitate the dialogue between patients and healthcare professionals in a mutually respectful manner to ensure harm reduction for patients and society.

Linear atrophoderma of Moulin (LAM) is an acquired skin condition that manifests in early childhood and adolescence. It likely represents a form of cutaneous mosaicism that presents with linear, hyperpigmented and atrophic lesions appearing on the trunk and limbs. Its clinical appearance varies and may closely resemble that of atrophoderma of Pasini and Pierini (APP) and linear scleroderma. LAM usually follows a benign course and no effective treatment options exist. We present a case of a young and healthy patient that developed such lesions on her upper and lower extremities over 5 years. The initial clinical impression of linear scleroderma was reviewed in favor of LAM following histological examination of the lesions which revealed no significant inflammatory changes. LAM remains a rare and possibly under recognized entity with reports confined only to the dermatologic literature. This case highlights the importance of recognizing LAM and distinguishing it from linear scleroderma given the significant differences in management and prognosis.

Objective To determine cancer incidence in a large clinical juvenile-onset arthritis population. Methods We combined data from 6 existing North American juvenile-onset arthritis cohorts. Patients with juvenile-onset arthritis were linked to regional cancer registries to detect incident cancers after cohort entry, defined as first date seen in the paediatric rheumatology clinic. The expected number of malignancies was obtained by multiplying the person-years observed (defined from cohort entry to end of follow-up) by the geographically matched age, sex and calendar year-specific cancer rates. The standardised incidence ratios (SIR; ratio of cancers observed to expected) were generated, with 95% CIs. Results The 6 juvenile arthritis registries provided a total of 5294 patients. The mean age at cohort entry was 8.9 (SD 5.0) years and 68% of participants were female. The mean duration of follow-up was 6.8 years with a total of 36 063 person-years spanning 1978–2012. During follow-up, 9 invasive cancers occurred, compared with 10.9 expected (SIR 0.82, 95% CI 0.38 to 1.5). 3 of these were haematological (Hodgkin9s, non-Hodgkin9s lymphoma and leukaemia). 6 of the patients with cancer were exposed to disease-modifying drugs; 5 of these had also been exposed to biological agents. Conclusions We did not clearly demonstrate an increase in overall malignancy risk in patients with juvenile-onset arthritis followed for an average of almost 7 years. 3 of the 9 observed cancers were haematological. 5 of the cancers arose in children exposed to biological agents. Longer follow-up of this population is warranted, with further study of drug effects.