混合性結合組織病

BACKGROUND: Regenerative therapies have become central to periodontics and implant dentistry and are becoming more widely applied to reconstruct supporting bone and associated soft tissues. With increased recognition of the benefits of using biomimetic materials as scaffolds in contemporary regenerative technologies, the field is seeking to develop and optimize predictable, personalized regenerative strategies for hard and soft tissue reconstruction. This review aims to: (1) evaluate the current evidence on these approaches in the context of hard and soft tissue deficiencies around teeth and implants, (2) assess the clinical utility of these approaches, and (3) propose a framework for integrating these regenerative technologies into daily clinical practice. METHODS: Applicable research on the treatment of hard and /or soft tissue deficiencies around teeth and dental implants were conducted. For soft tissue regeneration, data from eligible randomized clinical trials (RCTs) reporting the outcomes of gingival (for teeth, as gingival thickness [GT]) and mucosal thickness (implants, MT) with a bilaminar approach were analyzed using a mixed models network meta-analysis. A meta-analysis could not be performed to evaluate personalized, emerging technologies for bone regeneration given the lack of comparable clinical studies and as such, an alternative evaluative approach was undertaken and involved a qualitative assessment of the relevant literature highlighting innovations in these emerging modalities. RESULTS: A total of 78 RCTs were included for a quantitative analysis on soft tissue regeneration. Fifty-four reported the outcomes of GT with a bilaminar approach (a total of 2952 teeth), and 24 assessed MT around implants (851 implants). The analysis of GT for natural dentition showed that bilaminar approaches with any of the explored soft tissue grafts (connective tissue graft (CTG), human acellular dermal matrix (hADM), first generation collagen matrix (CMX), "volume stable" collagen matrix (VCMX) yield a statistically significant increase in GT (compared with untreated sites), with the highest estimate for CTG, followed by VCMX, hADM, pADM, and CMX. Similarly, the analysis of MT around implants also showed a significant increase for all the investigated treatment groups, compared with untreated sites. No other significant differences were noted among the other treatment groups of (VCMX, hADM, and pADM). For the qualitative assessment of emerging technologies for bone regeneration, early investigational studies have been conducted evaluating the use of computer aided personalized scaffold design and manufacturing, stem cell transplantation, stem cell secretome delivery, and artificial intelligence. To date, there are limited controlled studies evaluating these approaches but the results of studies which have been conducted demonstrate safety, efficacy, and favorable patient-reported outcomes. Clinical translation of these emerging, personalized technologies for hard tissue regeneration will largely depend upon feasibility and cost effectiveness. CONCLUSIONS: For the treatment of dento-alveolar deficiencies within the hard or soft tissue structures around teeth and implants, we found that autogenous soft tissue grafts as well as soft tissue grafting substitutes are generally effective to yield a significant increase in the soft tissue thickness of the treated sites. In the regeneration of hard tissue, emerging, personalized strategies have demonstrated promising early outcomes, yet additional longitudinal, controlled studies are required before these modalities can be considered for widespread clinical adoption. PLAIN LANGUAGE SUMMARY: Dentists and researchers are looking to find new ways to help the gums and bone around teeth and dental implants heal and grow back. In this review, we looked at 78 studies that tested different methods for improving gum tissue thickness as well as rebuilding the bone that supports teeth and implants. We found that using a patient's own gum tissue is still the most effective way to thicken and strengthen the gums. However, newer materials made from collagen or donated tissue can work almost as well and are easier for patients because they do not require tissue to be taken from the mouth. Around dental implants, all of these options helped increase gum tissue thickness, which may lower the risk of future disease and improve the look and long-term success of treatment. For rebuilding bone, early research on stem cells, 3D-printed materials, and products that help the body to heal on its own looks very promising, but larger studies are needed to confirm the results. Overall, this research shows that regenerative dentistry is moving toward more comfortable, less invasive, and more personalized treatments that can improve healing, appearance, and long-term stability.

Aortopathies in autoimmune rheumatic diseases (ARD) include a spectrum of aortic pathologies-including aortitis, aneurysms, dissections, and insufficiency-primarily caused by systemic inflammation. This comprehensive review investigates the clinical manifestations, pathophysiology, diagnostic modalities, and management strategies across various rheumatic diseases associated with aortopathies such as large vessel vasculitis (e.g., Takayasu arteritis, giant cell arteritis), connective tissue diseases (e.g., systemic lupus erythematosus, rheumatoid arthritis, ankylosing spondylitis, systemic sclerosis) and less common conditions (e.g., relapsing polychondritis, Cogan's syndrome, Behçet's disease, IgG4-related disease). Disease-specific pathophysiologic mechanisms of aortic wall inflammation and remodeling, including granulomatous and lymphoplasmacytic patterns and mixed inflammatory infiltrates, are described. Diagnostic imaging modalities-such as CTA, MRI, and PET/CT-are evaluated for their roles in detecting active inflammation, assessing structural complications, and guiding clinical decision-making. Histopathological findings provide insight into disease-specific vascular changes. Management strategies focus on the use of glucocorticoids, disease-modifying antirheumatic drugs (DMARDs), and biologics, including IL-6 and TNF-α inhibitors, with an emphasis on patient-centered approaches, multidisciplinary care, and timely surgical intervention for complications. Evidence gaps include optimal screening intervals and the role of novel biomarkers in risk stratification and in monitoring disease progression, highlighting the need for early recognition, frequent monitoring, and aggressive management of aortic involvement in rheumatic diseases to prevent life-threatening complications.

Leukocytoclastic vasculitis (LCV) is a small-vessel vasculitis that typically presents with palpable purpura on the lower extremities and is often confined to the skin. Peripheral nerve involvement is uncommon. We describe the case of a 17-year-old female who developed progressive burning paresthesias in all four limbs, asymmetric sensory loss, autonomic symptoms, and left foot drop preceding the appearance of palpable purpura and necrotic lesions on both lower limbs. Nerve conduction studies demonstrated mixed demyelinating and axonal motor neuropathy in both lower limbs, more pronounced on the left, with additional motor axonal involvement of the right median nerve. Sympathetic skin responses were absent in all four limbs. An extensive evaluation for infectious, connective-tissue disease-related, antineutrophil cytoplasmic antibody-associated, and paraproteinemic causes was unrevealing. Skin punch biopsy demonstrated dermal perivascular inflammation, leukocytoclasis, erythrocyte extravasation, and fibrinoid necrosis of small-vessel walls; direct immunofluorescence was positive for complement component 3 (C3), immunoglobulin M (IgM), and IgA. These findings were consistent with LCV. The patient was treated with intravenous methylprednisolone followed by oral corticosteroids and hydroxychloroquine, along with two doses of rituximab. The patient demonstrated marked improvement in left ankle dorsiflexion, with Medical Research Council (MRC) muscle power improving from 2/5 to 4+/5, and was able to walk independently without assistance. No new skin lesions developed. This report highlights that neurologic manifestations may precede the characteristic cutaneous eruption of LCV and that early electrophysiologic assessment and tissue biopsy can facilitate prompt diagnosis and treatment.

OBJECTIVE: This study aimed to explore evidence related to long-term health outcomes for patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (PAH) and any clinical practices associated with screening, early detection, and initiation of treatments. METHODS: We conducted a systematic literature review (SLR) of observational studies and randomised controlled trials of adult patients with confirmed CTD-PAH, including systemic lupus erythematosus (SLE)-PAH, systemic sclerosis (SSc)-PAH, and mixed CTD (MCTD)-PAH. Based on the findings from our SLR, we conducted a Delphi panel to develop recommendations and consensus around early detection and initiation of appropriate treatment for patients with CTD-PAH. RESULTS: Sixty-three publications (54 studies and 9 guidelines) were included in the SLR, with 22 involving Asia-Pacific populations. Combination therapy reduced clinical worsening and mortality versus monotherapy. After three Delphi rounds, consensus was reached on the importance of early PAH screening in CTD, with disease-specific recommendations for SLE, SSc, and MCTD. Consensus was also achieved on appropriate treatments, including endothelin receptor antagonists (ERA), phosphodiesterase-5 (PDE5) inhibitors, and pulmonary vasodilators. However, long-term outcomes remain uncertain due to limited evidence. CONCLUSION: This study highlights the need for early screening and timely treatment initiation in CTD-PAH to potentially reduce adverse long-term outcomes.

We report the case of a 30-year-old Somali female with mixed connective tissue disease (MCTD) who developed dermatomyositis and both scleroderma renal crisis (SRC) and fatal cardiac complications. Notably, she tested positive for both anti-U3 RNP and anti-RNA polymerase III antibodies which is a rare serologic combination. After initial treatment with corticosteroids for dermatomyositis features, she experienced rapid clinical deterioration marked by malignant hypertension and thrombotic microangiopathy and was found to have SRC and diffuse myocardial fibrosis. Despite treatment with captopril and hemodialysis, she ultimately died of cardiac arrest. This case highlights the complexity and risks in managing patients with overlapping autoantibodies and underscores the need for close monitoring and cautious use of steroids in such high-risk individuals.