Interstitial lung disease (ILD) can lead to lung cancer, which brings great challenges to differential diagnosis and comprehensive treatment. However, the clinical features of lung-dominant connective tissue disease (LD-CTD) related ILD combined with lung cancer has not been validated. We report the case of an 80-year-old woman with LD-CTD treated regularly with nintedanib who presented progressive dyspnoea and hypoxemia after recurrent viral infections. Her chest computed tomography (CT) showed aggravated interstitial fibrosis in both lower lungs with moderate right pleural effusion. Clinicians should be alert to lung cancer in patients who are experiencing poor responsiveness to treatment or acute progression of ILD. The available literatures about the differential diagnosis of clinical manifestations, imaging, treatment and prognosis of LD-CTD are reviewed and discussed in this study.

OBJECTIVE: Loeys-Dietz syndrome (LDS) is a rare, autosomal dominant connective tissue disorder which can aggressively affect the aortic vasculature. Limited information is available regarding its impact on pregnancy and postpartum outcomes.
METHODS: A pregnant 38-year-old nulliparous woman with mild aortic regurgitation and family history of aortic aneurysms presented with an aortic root measuring 49 mm. Despite concerns of an underlying connective tissue disorder, a definitive diagnosis was not reached. She delivered under strict blood pressure control, developed intractable uterine atony, and underwent uterine artery embolization. On the second postpartum day, aortic dissection was incidentally diagnosed, and aortic root replacement surgery was performed. Genetic testing revealed a novel in-frame SMAD3 deletion [NM_005902.4: c.703_708del, (p.Ile235_Ser236del)], leading to a diagnosis of LDS type 3.
CONCLUSIONS: This case highlights the high postpartum aortic dissection risk in women with LDS, emphasizing the importance of early diagnosis in pregnant women with few clinical symptoms.

BACKGROUND: The primary therapies for connective tissue disease include glucocorticoids and immunosuppressants. However, their prolonged usage can precipitate opportunistic infections, such as cytomegalovirus infection. When managing connective tissue disease complicated by cytomegalovirus infection, judicious selection of treatment modalities is crucial. This involves assessing the necessity for antiviral therapy and contemplating the reduction or cessation of glucocorticoids and immunosuppressants.
OBJECTIVE: This investigation sought to methodically review existing literature regarding treating connective tissue disease patients with cytomegalovirus infection.
METHODS: On July 5, 2023, an exhaustive literature search was conducted. Data analysis utilized the Kruskal-Wallis test or one-way analysis of variance, supplemented by Bonferroni post hoc testing.
RESULTS: Our meta-analysis incorporated 88 studies encompassing 146 connective tissue disease patients with CMV infections. The results indicated that patients with connective tissue disease and cytomegalovirus disease benefitted more from antiviral therapy than those not receiving such treatment (P = 0.003, P < 0.005). Furthermore, the strategic reduction of glucocorticoids and/or immunosuppressants was beneficial (P = 0.037, P < 0.05). Poor clinical outcomes with glucocorticoid-immunosuppressant combination therapy compared to other treatment modalities. The findings also suggested that CMV infection patients fare better without Cyclosporine A than using it (P = 0.041, P < 0.05).
CONCLUSIONS: Antiviral therapy is a viable treatment option in cases of connective tissue disease co-occurring with cytomegalovirus disease. Additionally, when connective tissue disease is stable, there is potential merit in reducing glucocorticoids and/or immunosuppressants, especially avoiding the combination of these drugs. For all cytomegalovirus infection patients, Cyclosporine A may be avoided wherever possible for selecting immunosuppressive agents if its use is not deemed essential in the treatment regimen.

BACKGROUND: Connective tissue disease (CTD) is a heterogeneous group of chronic inflammatory autoimmune disorders derived from a systemically auto-immunological deregulation. CTD may affect cardiac structures through multiple pathophysiological mechanisms, and subclinical cardiac injury is common. Heart failure (HF) is one of the common complications in these patients.
METHODS: Patients with CTD suffer an increased risk of cardiovascular disease and may have chest pain and shortness of breath.
METHODS: HF is characterized by dyspnea or exertional limitation due to impaired ventricular filling and/or blood ejection. HF can be caused by other systemic diseases, not only by cardiovascular disorders but CTD. CTD may cause HF due to diffuse myocardial damage, heart valve damage, coronary ischemia, and so on.
METHODS: The patient with catastrophic antiphospholipid syndrome take prednisone and warfarin. The patient with anti-synthetase syndrome was treated with immunoglobulin, followed by long-term oral medicines of prednisone, methotrexate, and folic acid.
RESULTS: The symptoms of chest pain and shortness of breath for patients with CTD improved.
CONCLUSIONS: HF is one of the common complications in these patients with CTD, which has poor prognosis and severe aggravation. Once such patients experience chest pain, chest tightness, shortness of breath, etc, we should consider the possibility of HF. Early identification and correct treatment can delay the progression of HF, improve the prognosis, and enhance the quality of life for patients. Therefore, we should pay more attention to patients with CTD combined with HF.

Pulmonary arterial hypertension (PAH), idiopathic pulmonary fibrosis (IPF), and scleroderma (SSc) are three interrelated medical conditions that can result in significant morbidity and mortality. Pulmonary hypertension, a condition marked by high blood pressure in the lungs, can lead to heart failure and other complications. Idiopathic pulmonary fibrosis, a progressive lung disease characterised by scarring of lung tissue, can cause breathing difficulties and impaired oxygenation. Scleroderma, an autoimmune disease, can induce thickening and hardening of the skin and internal organs, including the lungs, leading to pulmonary fibrosis and hypertension. Currently, there is no cure for any of these conditions. However, early detection and proper management can improve the quality of life and prognosis of a patient. This review focusses on PH and IPF in patients with SSc, providing information on the causes, symptoms, and treatment of these conditions, together with illustrative images. It also provides an overview of interrelated medical conditions: PH, IPF, and SSc. It emphasises the importance of early detection and proper management to improve patient quality of life and prognosis.

暂无摘要。

BACKGROUND: Multisystemic inflammatory syndrome is a syndrome of multisystem involvement caused by a novel coronavirus infection that can lead to cardiogenic shock and death in children.
METHODS: A 4-year-old girl was diagnosed with multiple organ and multiple system involvement after Kawasaki disease.
METHODS: Novel coronavirus infection-associated multisystem inflammatory syndrome in children was considered.
METHODS: The patients received aspirin, methylprednisolone and gammaglobulin to treat multisystem inflammatory syndrome.
RESULTS: After treatment, the child recovered and was discharged from the hospital.
CONCLUSIONS: Multisystem inflammatory syndrome is often mistaken for Kawasaki disease, fortunately, their treatments are similar, the purpose of this case is to remind clinicians of the need for early management of children with multisystem failure following novel coronavirus infection, increase the detection rate, and save the life of the child.

暂无摘要。

Hereditary connective tissue disorders are a broad group of congenital disorders that are characterized by a pathological weakness of the connective tissue as a result of an incorrect genesis, leading to multisystem complaints. We describe a 14-year-old patient with the hereditary connective tissue disorder Loeys-Dietz syndrome who was admitted to a child psychiatric crisis unit because of depressive and anxiety symptoms. A systematic literature search was carried out to analyze the prevalence of depressive and anxiety symptoms in individuals with hereditary connective tissue disorders Loeys-Dietz syndrome, Ehlers-Danlos syndrome and Marfan syndrome, to identify a possible association between these disorders and explanations for this. We conclude that there is an increased incidence of depression and anxiety symptoms in which pain, fatigue, social support and functioning, quality of life and functional limitations seem to play a role. There is a need for further research to determine exactly which factors contribute and how these can be targeted in prevention and treatment.

A 37-year-old woman was hospitalized with fever and consciousness disturbance. She showed systemic inflammation with stress cardiomyopathy. Brain computed tomography showed diffuse brain edema. Cerebrospinal fluid (CSF) findings revealed markedly elevated cerebrospinal fluid pressure with pleocytosis, elevated protein, and elevated interleukin 6. The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) nicking enzyme amplification reaction test using a nasopharyngeal swab was positive, and the patient was diagnosed with SARS-CoV-2 infection. From the negative result of the CSF SARS-CoV-2 polymerase chain reaction test and no findings of bacterial or viral infection, we diagnosed meningoencephalitis by multisystem inflammation syndrome in adults (MIS-A). Intravenous methylprednisolone pulse therapy improved her symptoms and brain edema. There have been no cases of MIS-A with meningoencephalitis, and no initial treatment strategy has been established, especially in emergency cases of suspected MIS-A. The present case suggested Early intravenous methylprednisolone pulse with anti-coronaviral therapies after the exclusion of bacterial infection would be useful in suspected MIS-A with emergent meningoencephalitis cases.