Isolated Rosai-Dorfman disease of the spine: A systematic literature review.

INTRODUCTION: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis involving the central nervous system in 5% of cases. Spinal location occurs in less than 1% of extranodal RDD and can be responsible for neurological manifestations. We present a systematic review of cases of isolated spinal RDD. We also report a new case of isolated spinal RDD revealed by spinal cord compression. MATERIALS AND METHODS: The systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guideline using the MEDLINE and SCOPUS databases and included case reports and case series describing isolated RDD of the spine. RESULTS: There were 53 patients with isolated spinal RDD (including our case). The mean age was 35.85±16.48 years. Neurological deficit was the most frequent clinical presentation (89%). RDD lesions were mainly located in the thoracic spine (51%), then the cervical spine (32%). The lesion was reported to be extradural (57%), intradural extramedullary (26%), intramedullary (7%), and in the vertebral body (10%). Histological examination showed emperipolesis in 73%. Histocytes were positive for S-100 protein in 83%. Treatment was based on surgery 96%), radiotherapy, chemotherapy, and adjunctive steroid therapy were indicated in four, one, and eight cases. After a mean follow-up period of 14.84±13.00 months, recurrence of RDD was noted in 15%. CONCLUSION: Spinal RDD is a rare condition, requiring meticulous histological examination for accurate diagnosis. Complete surgical resection is the treatment of choice. Adjuvant chemotherapy and radiotherapy can also be indicated in patients demonstrating partial improvement following surgery.

COVID-19 in Patients with Systemic Inflammatory Diseases: Impact on Disease Activity.

INTRODUCTION: COVID-19 pandemic, an international emergency, raised concerns about the interaction of this infection and disease-modifying drugs used in the treatment of Systemic inflammatory diseases (SID). Understanding the relationship between COVID-19 and disease activity is crucial to adapt the treatment. AIM: The aim of our study was to determine the impact of COVID-19 on the disease activity of rheumatic diseases. PATIENTS AND METHODS: We performed a cross-sectional study, including patients with SID (rheumatoid arthritis (RA) and spondyloarthritis (SpA)). Disease activity was evaluated during the last check-up before COVID-19 and within the period of 6 months after the infection. Activity scores were assessed with Disease Activity Score (DAS28) for RA and Ankylosing Spondylitis Disease Activity Score (ASDAS) for SpA. Correlation and regression coefficients were used to evaluate associations among the variables. RESULTS AND DISCUSSION: Totally, thirty-two patients were included; twenty followed for RA and twelve for axial SpA. The mean disease duration of the underlying rheumatic disease was 10.2 years (2-30). RA was seropositive and erosive in 61% and 31%, respectively. Seventeen patients were on csDMARDs: 14 were on Methotrexate and three patients were on Salazopyrine. Ten patients (31%) were treated with bDMARDs; Tumor necrosis factor (TNF)-alpha inhibitors were used in eight cases. Rituximab and secukinumab were prescribed for one patient each. In 70%, COVID-19 was pauci-symptomatic. A severe form with a need for hospitalization was noted in 9%. Two patients were admitted to the intensive care unit (ICU). Overall, treatment with DMARDs was interrupted in all cases: when COVID-19 symptoms began in 82% and when PCR was positive in 18%. Both RA and axial SpA were not active after a mean period of 6 months after COVID-19 infection (p = 0.818 and p = 0.626, respectively). CONCLUSION: Although our patients interrupted their DMARDs, our study demonstrates that disease activity as assessed by ASDAS and DAS28 in SpA and RA remained unchanged after COVID-19.

Matrix Metalloproteinases; A Biomarker of Disease Activity and Prognosis in Spondyloarthritis: A Narrative Review.

BACKGROUND: Matrix metalloproteinases, as components of the proteolytic system, are deemed to be implicated in the pathogenesis and progression of several rheumatic diseases. Their role in spondyloarthritis has been investigated by several studies. OBJECTIVE: This article aims to review and summarize the current knowledge related to metalloproteinases in patients with spondyloarthritis. METHODS: To examine the association between matrix metalloproteinases and spondyloarthritis, we conducted a narrative review using a literature search in SCOPUS for English-language sources. The search included studies published from the database inception to December 2020. RESULTS: A total number of 74 articles were included. It was found that levels of matrix metalloproteinases 3 were higher in radiographic axial spondyloarthritis patients and seemed to play a role in the progression of joint damage. The levels of matrix metalloproteinases 1, 2, and 9 were upregulated in psoriatic arthritis patients compared to psoriasis and could identify psoriasis patients who would develop rheumatic manifestations. The levels of matrix metalloproteinases correlated significantly with disease activity in ankylosing spondylitis and decreased upon treatment with Tumor Necrosis Factor inhibitors (TNFi). CONCLUSION: Excessive matrix metalloproteinases activity is associated with articular destruction. Their levels can reflect disease activity, structural damage, and response to TNFi in patients with spondyloarthritis. Nevertheless, further studies are needed to confirm these results.