Articles: connective-tissue-diseases.
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Internal medicine journal · Oct 2023
ReviewMultidisciplinary team discussion: the emerging gold standard for management of cardiopulmonary complications of connective tissue disease.
Cardiopulmonary complications of connective tissue diseases (CTDs), particularly pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD), are major determinants of morbidity and mortality. Multidisciplinary meetings may improve diagnostic accuracy and optimise treatment. We review the literature regarding multidisciplinary meetings in CTD-ILD and PAH and describe our tertiary centre experience of the role of the multidisciplinary meeting in managing CTD-PAH.
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Multisystem inflammatory syndrome in adults (MIS-A) is a life-threatening disease that can develop weeks after coronavirus disease 2019 (COVID-19). MIS-A symptoms include multiorgan involvement, especially gastrointestinal tract and heart involvement, and Kawasaki disease-like symptoms. ⋯ Methylprednisone pulse and high-dose intravenous immunoglobulin resulted in recovery of shock and his renal function, but diffuse ST-segment elevation on electrocardiography and pericardial effusion with a fever emerged after therapy. Additional granulocyte-monocyte adsorptive apheresis successfully ameliorated the cardiac involvement.
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A 21-year-old man on hemodialysis was hospitalized for coronavirus disease 2019 (COVID-19) pneumonia. After admission, he had a persistent high fever and developed erythema induratum on his extremities. Laboratory tests conducted 25 days after onset showed markedly increased procalcitonin (PCT) levels (>100 ng/mL). ⋯ Based on the presence of erythema induratum and multiorgan dysfunction, he was diagnosed with multisystem inflammatory syndrome (MIS). The MIS resolved after treatment with intravenous immunoglobulin and methylprednisolone. This report illustrates that MIS can occur in adults and may be accompanied by high PCT levels.
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Systemic sclerosis (SSc) is a rare auto-immune disease, affecting principally women between 40 and 60 years old. It is caracterised by a cutaneous and visceral fibrosis, an alteration of the microvascular network and the presence of autoantibodies. SSc can be associated with another connectivite tissue disease or to other autoimmune diseases, thus defining the overlap syndrome. The goal of our study is to describe these overlap syndromes. ⋯ SSc is often associated with other autoimmune diseases. This interrelation between associated pathologies and SSc, modifying sometimes the evolution of SSc, enhances the need of a personalized follow-up.