OBJECTIVES:To report pregnancy outcome and neonatal immune parameters following early conception after maternal CD19 chimeric antigen receptor T-cell (CAR-T) therapy for refractory systemic lupus erythematosus (SLE). METHODS:Maternal and neonatal CAR-T cells were assessed by transgene polymerase chain reaction (PCR) at delivery. CD19+ B-cell counts and immunoglobulin levels were measured in maternal and neonatal blood at birth. Infant health outcomes were assessed during the first year of life. RESULTS:Conception occurred approximately seven weeks after CD19 CAR-T infusion. CAR-T cells were not detected in maternal peripheral blood or cord blood at delivery. Neonatal CD19+ B-cell counts and IgG levels were within age-appropriate reference ranges and exceeded maternal values, which remained subnormal. During one year of follow-up, the infant experienced no recurrent or severe infections. CONCLUSIONS:This case represents the earliest reported pregnancy following CD19 CAR-T therapy for SLE and the first to include neonatal immune assessment at birth. No evidence of transplacental CAR-T transfer or clinically significant neonatal immunodeficiency was observed. Additional cases and long-term follow-up are required to guide reproductive counseling after CAR-T therapy.

Retroperitoneal fibrosis (RPF) and sclerosing mesenteritis (SM) are rare entities characterized by fibrous inflammatory tissue that can surround and compress adjacent abdominal structures, leading to organ damage. RPF and SM have rarely been reported to present simultaneously. Idiopathic forms of RPF and SM are typically treated with immunosuppression, whereas treatment of RPF and SM secondary to malignancy relies on treating the underlying malignancy. We present a patient with steroid-responsive RPF and SM ultimately found to be secondary to an occult small intestinal neuroendocrine tumor who responded to octreotide therapy for the underlying neuroendocrine tumor.

Immune checkpoint inhibitors have become the cornerstone in the management of a variety of cancers and have been implicated in immune-related rheumatological adverse events, including inflammatory myositis, polyarthritis and vasculitis. Existing literature has documented large vessel vasculitis in association with ipilimumab, pembrolizumab and nivolumab therapy. Although large vessel vasculitis was reported in one patient who had received both atezolizumab and pegfilgrastim, our patient represents the first instance of large vessel vasculitis associated with atezolizumab alone. Discontinuation of atezolizumab and tapered glucocorticoid therapy resulted in clinical and radiographic resolution.

Clinically significant granulomatous inflammation of skeletal muscle in sarcoidosis is rare. Glucocorticoids are generally considered the first-line treatment of sarcoidosis, but due to their side effect profile, the addition of steroid-sparing regimens has become increasingly more common. We report a patient with nodular sarcoid myositis who was successfully treated with antimalarial hydroxychloroquine alone. Whereas antimalarials have been reported to be an effective treatment of various organ involvement in sarcoidosis, to our knowledge, this is the first report of hydroxychloroquine monotherapy successfully treating nodular sarcoid myositis. Hydroxychloroquine monotherapy may be a reasonable initial treatment option for nodular sarcoid myositis and other forms of muscular sarcoidosis, as well as for other non-acute organ-threatening manifestations of the disease.

Extraintestinal manifestations (EIMs) are frequent complications of the classical inflammatory bowel diseases, Crohn's disease and ulcerative colitis. However, in addition to the classical diseases, there is a spectrum of conditions, often termed 'microscopic colitis' (MC), in which EIMs are less well described. Our objective was to review the literature regarding the EIMs complicating MC and describe their association with systemic autoimmune rheumatic diseases. A comprehensive search and review of peer-reviewed English-language and international journals and reports was completed based on key terms, including 'microscopic colitis', 'lymphocytic colitis', 'collagenous colitis', 'inflammatory bowel disease', and 'extraintestinal manifestations', and the specific disease associations utilizing the PubMed Central database and MEDLINE. A broad spectrum of rheumatologic manifestations has been reported in patients with MC. The identification of rheumatoid arthritis and limited scleroderma as comorbidities with MC was noteworthy. Inflammatory arthropathy was frequently seen in MC, usually preceding or occurring in conjunction with the onset of gastrointestinal symptoms. A variety of presentations of associated arthritis were reported: migratory, symmetric or asymmetric, peripheral or axial, oligoarticular or polyarticular, and erosive or non-erosive. There was a high incidence of autoantibodies in these patients, supporting a potential autoimmune association. On the basis of these anecdotal reports, we would suggest the development of a clinical registry to help define the incidence of EIMs and systemic autoimmune rheumatic diseases among MC patients to help elucidate shared predispositions, pathogenic mechanisms, and therapeutic opportunities.

Although autoimmune manifestations can be associated with various lymphomas, they are distinctly unusual with primary cutaneous anaplastic large cell lymphoma (PCALCL). We present the case of a 76-year-old woman who, over the course of 2 years, presented with a variety of autoimmune disorders including minimal change disease and focal glomerulosclerosis, visual loss due to retinal vasculitis, immune mediated thrombocytopenia, autoimmune hemolytic anemia, and inflammatory polyarthritis in conjunction with elevated rheumatoid factor, cryoglobulins, and hypocomplementemia. She ultimately developed PCALCL that took an aggressive course and to which she ultimately succumbed. Our case adds to the growing literature demonstrating autoimmune manifestations associated with a variety of lymphoid malignancies. We propose that immune dysregulation can predispose patients to the development of both autoimmune and lymphoproliferative disease. Suspicion of underlying malignancy in patients presenting with otherwise unexplained autoimmune phenomena may prompt earlier diagnosis.

The objective of this study was to compare rheumatoid arthritis (RA) disease activity and patient-reported outcomes (PROs) in a national sample of patients with RA with/without Sjögren’s syndrome (SS). Adults with RA from a large observational US registry (Corrona RA) with known SS status between 22 April 2010 and 31 July 2018 and a visit 12 (± 3) months after index date were identified (n = 36,256/52,757). SS status: determined from a yes/no variable reported at enrolment into the Corrona RA registry and follow-up visits. Index date: date that SS status was recorded (yes/no). Patients received biologic or targeted synthetic disease-modifying antirheumatic drugs as part of standard care. Patients with RA only were followed for ≥ 12 months to confirm the absence of SS. Patients were frequency- and propensity-score matched (PSM) 1:1 and stratified by disease duration and treatment response-associated variables, respectively. Clinical Disease Activity Index (CDAI) and PROs 12 months after index visit were compared in patients with and without SS. Baseline characteristics in 283 pairs of PSM patients were balanced. Mean change in CDAI score was numerically lower in patients with RA and SS than patients with RA only (8.8 vs 9.3). Reductions in PROs of pain, fatigue and stiffness were two- to threefold lower for patients with RA and SS versus RA only. Reductions in RA disease activity and RA-related PROs were lower in patients with RA and SS versus those with RA only. Our data indicate that SS adds to treatment challenges; physicians may wish to consider SS status when managing patients with RA.

The objectives of this analysis were to assess the prevalence of Sjögren’s syndrome (SS) associated with rheumatoid arthritis (RA) and to compare baseline characteristics of patients with RA with and without SS. Adult patients with RA from a large observational US registry (Corrona RA), with ≥ 1 visit for assessment of SS status between 22 April 2010 and 28 February 2018, were considered. Patients with RA with versus without SS were compared. SS status was determined from a yes/no variable and reported at enrollment into the Corrona RA registry and follow-up visits. Outcomes were unadjusted prevalence of SS in patients with RA, prevalence of SS by RA disease duration, and baseline characteristics in patients with RA by SS status. Of 24,528 eligible patients, 7870 (32.1%) had a diagnosis of RA and SS. The unadjusted overall rate for SS prevalence in patients with RA was 0.30 (95% confidence interval 0.29, 0.31). SS prevalence increased with increasing RA duration. Patients with RA with versus without SS were more likely to be older, female, and seropositive; had a longer RA duration; higher disease activity; and a higher incidence of comorbidities (hypertension, cardiovascular disease, malignancies, and serious infections), erosive disease, and subcutaneous nodules at index date. Patients with RA and SS had a higher disease burden than those with RA only. The prevalence of SS increased as duration of RA increased. RA with SS was associated with seropositivity, more severe RA, extra-articular manifestations, and comorbidities. Key Points • The overall prevalence of SS among patients with RA was 30%. • The prevalence of SS increased with increasing RA disease duration. • Identifying specific clinical characteristics of patients with RA with SS, such as a greater incidence of extra-articular manifestations and comorbidities, may help clinicians to better characterize this patient population.

Human parvovirus B19 (B19V) is well known for its infectivity. However, the risk for communicability to previously unexposed healthcare professionals is controversial. We report here a small outbreak of B19V infection among physicians and family members in an adult rheumatology practice that occurred after providing care for a patient with B19V arthropathy. As B19V-infected patients who demonstrate findings of erythema infectiosum or viral arthritis are generally beyond the period of transmissability, strict handwashing and droplet precautions remain imperative when there is contact with potentially pre-symptomatic family members.

Malignant catatonia represents a potentially life-threatening presentation of catatonia that typically includes fever, autonomic instability, and cardiovascular collapse. Failure to respond to benzodiazepines, particularly with an increase in catatonic signs, should raise suspicion for the presence of malignant catatonia. Here, we report the first case of malignant catatonia as the initial manifestation of systemic lupus erythematosus (SLE) presenting in a 15-year-old girl who developed progressive catatonia that was resistant to immunosuppressive therapy with pulse corticosteroids, intravenous immunoglobulin (IVIg), and cyclophosphamide and required electroconvulsive therapy for clinical improvement. Catatonia in SLE is reviewed and contrasted with other types of autoimmune encephalopathies, anti-NMDAR antibody encephalitis, and Hashimoto encephalopathy (HE), highlighting the diagnostic considerations and clinical challenges.

Background Sjögren’s syndrome (SS) is known to occur secondary to RA diagnosis, but its impact on RA outcomes is poorly understood. In the US-based Corrona registry, SS has a prevalence rate of 0.30 in patients (pts) with RA.1 Objectives To compare disease activity and patient-reported outcomes (PROs) in pts with and without SS, in a national sample of pts with RA. Methods We identified adult pts with rheumatologist-diagnosed RA from a large observational US registry (Corrona RA), with at least one visit with known SS status between 22 Apr 2010 and 31 Jul 2018 and a visit 12 (±3) months after index date. SS status was captured using physician forms at enrollment and follow-up visits. The index date was the date of first capture of SS diagnosis, or first visit in which SS status was recorded for pts without a diagnosis of SS (non-SS pts). Both those with and without SS were required to have initiated a targeted synthetic (ts) or biologic (b)DMARD. Non-SS pts had to be enrolled for ≥12 months. Pts were frequency matched 1:1 based on RA duration prior to 1:1 propensity score matching (PSM) based on variables associated with treatment response. Outcomes 12 months after index visit were compared in pts with and without SS. Primary outcome was mean change in CDAI score from index visit to Month 12; secondary outcome was mean change in PROs (pain, fatigue, pt global assessment and morning stiffness). Results After PSM, baseline characteristics in 283 pairs of pts with and without SS were well balanced (Table 1). Mean change in CDAI score was numerically lower in SS than non-SS pts (8.8 vs 9.3; Table 2), but this did not reach statistical significance. Improvements in pain, fatigue and stiffness were 2–2.5-fold greater for non-SS than SS pts (Table 2). Conclusion Among those initiating b/tsDMARD treatment, improvements in RA-related PROs were lower in pts with RA with SS than non-SS pts. Physicians may want to consider SS status secondary to RA when managing pts with RA and specifically explore whether additional intervention is needed to improve the pt experience for pts with RA. References [1] Harrold LR, et al. Arthritis Rheumatol. 2018;70(suppl 10): abstract 541. Acknowledgement This study was sponsored by Corrona, LLC. Corrona is supported through contracted subscriptions with multiple pharmaceutical companies. The abstract was a collaborative effort between Corrona and Bristol-Myers Squibb (BMS) with financial support provided by BMS. Professional medical writing: Lola Parfitt, MRes, Caudex; funding: BMS Disclosure of Interests Leslie R Harrold Shareholder of: Corrona LLC, Grant/research support from: Grant funding: Pfizer, Consultant for: AbbVie, Bristol-Myers Squibb, Genentech, Employee of: Corrona LLC, Ying Shan Employee of: Corrona LLC, Sabrina Rebello Employee of: Corrona, LLC, Neil Kramer Shareholder of: Johnson & Johnson, Pfizer, Regeneron, AbbVie, Amgen, Sean Connolly Shareholder of: Bristol-Myers Squibb, Employee of: Bristol-Myers Squibb, Evo Alemao Shareholder of: Bristol-Myers Squibb, Employee of: Bristol-Myers Squibb, Sheila Kelly Shareholder of: Bristol-Myers Squibb, Employee of: Bristol-Myers Squibb, Joel Kremer Grant/research support from: AbbVie, Genentech, Lilly, Novartis, Pfizer, Consultant for: AbbVie, Amgen, BMS, Genentech, Lilly, Regeneron, Sanofi, Pfizer, Elliot D Rosenstein Grant/research support from: AbbVie, Novartis, Consultant for: Amgen, Bristol-Myers Squibb, Boehringer Ingelheim, Speakers bureau: Amgen, Bristol-Myers Squibb

At the intersection of atopy and autoimmunity, we present a patient with seronegative rheumatoid arthritis (RA) who developed hypereosinophilia, without evidence of other etiologies, as she became rheumatoid factor (RF) positive. Although the magnitude of eosinophilia in patients with RA has been thought to reflect the severity or activity of the RA, in our patient, eosinophilia developed at a time when the patient's synovitis was well controlled. Although eosinophilia may reflect associated drug hypersensitivity, discontinuation of the medications utilized to control our patient's disease, adalimumab and methotrexate, did not promote clinical improvement. Probably the most curious aspect of our patient was the concomitant development of rheumatoid factor seropositivity in the setting of previously seronegative RA. The temporal relationship between the development of peripheral eosinophilia and seroconversion suggests a possible connection between these events. We speculate that the T cell cytokine production that can induce eosinophilia may simultaneously activate RF production.

Despite advances in our understanding of the inflammatory events that underlie rheumatoid arthritis (RA), which have led to targeted therapies that more effectively control the condition, the etiology of RA is not fully understood. With the discovery that serum antibodies to citrullinated peptides (ACPA) are highly specific for RA and that Porphyromonas gingivalis, the major pathogen responsible for periodontitis (PD), contains the enzyme responsible for the citrullination of peptides, a plausible explanation for observations of increased incidence and severity of PD in RA patients and an appreciation of pathogenic similarities between the two conditions has emerged. Studies of the effect of RA treatment on the severity of PD have been limited and conflicting, especially with respect to anti-TNF agents, but indicate the potential for IL-6 as a therapeutic target for both conditions. PD treatment appears to improve clinical and laboratory evidence of RA disease activity, and the response of RA to anti-TNF therapy is abrogated by the presence of PD. Thus, evaluation and treatment of PD can be recommended for all RA patients.