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Symmetric inflammatory polyarthritis is the most prominent manifestation of rheumatoid arthritis (RA). However, RA can practically affect any organ system, whether hematologic, neurological, cardiac, lung, skin, eyes, or kidneys. Systemic involvement in RA can be severe when there is interstitial lung disease, scleritis, amyloidosis, pure red cell aplasia, or myelodysplasia. Cardiovascular disease is the leading cause of death in patients with RA with a similar cardiovascular risk to that with diabetes mellitus. Patients with RA are at an increased risk of infections or osteoporosis, largely due to treatment-related etiologies. Rheumatoid vasculitis is a devastating long-term complication of RA which is fortunately becoming rarer over time due to better disease activity control. While the risk of mortality overall seems to be reducing over time, the excess mortality risk with RA compared with the general population persists. Fibromyalgia, anxiety, depression, fatigue, and physical inactivity remain important comorbidities associated with RA., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

We describe a fatal case of diffuse alveolar hemorrhage (DAH) complicated by rheumatoid arthritis (RA). A female patient was diagnosed with RA two months earlier and was treated with prednisolone and tacrolimus due to abnormalities in chest images. The patient was admitted to Hamanomachi Hospital for exertional dyspnea and was treated for exacerbation of chronic heart failure. Even after treatment for heart failure, exertional dyspnea remained. Chest CT imaging revealed contractile, patchy consolidations and ground-glass opacities (GGO) with a peribronchial distribution, suggesting an organizing pneumonia (OP) pattern. She was then treated with an additional 25 mg/day of prednisolone following a clinical diagnosis of OP. When the prednisolone dose was tapered, chest imaging showed worsening infiltration. A bronchoscopy was conducted, and bronchoalveolar lavage fluid was sanguineous, indicating DAH. Given that additional workup for the other etiology of DAH was negative, DAH was thought to be related to RA. Intensive treatment, including pulse dose methylprednisolone, failed to halt progression of respiratory failure, leading to a fatal outcome. The clinical presentation proved challenging due to its rarity. DAH might be a differential diagnosis in RA patients with consolidations and GGO in chest CT images. We review past cases of RA-associated DAH and assess potential treatment choices for future cases.

Background: Significant changes in the epidemiology and natural history of rheumatoid vasculitis (RV) have occurred with the introduction of biological therapies such as TNF inhibitors (TNFi) and rituximab., Purpose: This scoping review aims to address the key current challenges and propose updated criteria for RV. This will aid future descriptive observational studies and prospective therapeutic trials., Methodology: The MEDLINE database was searched for eligible articles from inception through December 2022. Articles were selected based on language and publication date after 1998, corresponding to the approval of the first TNFi in rheumatic diseases., Results: Sixty articles were included in the review. The mean incidence of RV has decreased since the approval of biologic therapies in RA, from 9.1 (95% CI: 6.8-12.0) per million between 1988 and 2000 to 3.9 (95% CI: 2.3-6.2) between 2001 and 2010, probably due to significant improvement in RA severity and a decrease in smoking habits. Factors associated with an increased risk of RV include smoking at RA diagnosis, longer disease duration, severe RA, immunopositivity, and male gender (regardless of age). Homozygosity for the HLA-DRB104 shared epitope is linked to RV, while the presence of HLA-C3 is a significant predictor of vasculitis in patients without HLA-DRB104. Cutaneous (65-88%), neurologic (35-63%), and cardiac (33%) manifestations are common in RV, often associated with constitutional symptoms (70%). Histologic findings range from small vessel vasculitis to medium-sized necrotizing arteritis, but definite evidence of vasculitis is not required in the 1984 Scott and Bacon diagnostic criteria. Existing data on RV treatment are retrospective, and no formal published guidelines are currently available., Conclusion: The understanding of RV pathogenesis has improved since its initial diagnostic criteria, with a wider range of clinical manifestations identified. However, a validated and updated criteria that incorporates these advances is currently lacking, impeding the development of descriptive observational studies and prospective therapeutic trials., Primary Funding Source: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors., Competing Interests: Declaration of Competing Interest Laurent ARNAUD is a consultant for Alexion, Amgen, Astra-Zeneca, Abbvie, Biogen, BMS, Boehringer-Ingelheim, Cêmka, GSK, Janssen, Kezar, LFB, Lilly, Menarini France, Medac, Novartis, Oséus, Pfizer, Roche-Chugaï, Sêmeia, UCB., (Copyright © 2023 Elsevier B.V. All rights reserved.)

The autoimmune peripheral neuropathies with prominent motor manifestations are a diverse collection of unusual peripheral neuropathies that are appreciated in vast clinical settings. This chapter highlights the most common immune-mediated, motor predominant neuropathies excluding acute, and chronic inflammatory demyelinating polyradiculoneuropathy (AIDP and CIDP, respectively). Other acquired demyelinating neuropathies such as distal CIDP and multifocal motor neuropathy will be covered. Additionally, the radiculoplexus neuropathies, resulting from microvasculitis-induced injury to nerve roots, plexuses, and nerves, including diabetic and nondiabetic lumbosacral radiculoplexus neuropathy and neuralgic amyotrophy (i.e., Parsonage-Turner syndrome), will be included. Finally, the motor predominant peripheral neuropathies encountered in association with rheumatological disease, particularly Sjögren's syndrome and rheumatoid arthritis, are covered. Early recognition of these distinct motor predominant autoimmune neuropathies and initiation of immunomodulatory and immunosuppressant treatment likely result in improved outcomes., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Neurologic manifestations of rheumatoid arthritis (RA) range in severity from mild paresthesias in the hand from carpal tunnel syndrome to sudden death due to impingement of the medulla by an eroded, vertically subluxed dens. Most neurologic complications are a consequence of articular inflammation and damage that leads to compression of adjacent structures of the central or peripheral nervous systems. Rare but serious extra-articular manifestations include inflammation of the meninges and ischemic neuropathies due to necrotizing arteritis of the vasa vasorum. Medical therapy with synthetic disease-modifying antirheumatic drugs and biological agents has diminished the incidence of serious neurologic manifestations in RA., (Copyright © 2017 Elsevier Inc. All rights reserved.)