Severe Pulmonary Hypertension Due to Adult-Onset Still’s Disease

Sinha, Ankur; Patti, Ravikaran; Ambesh, Paurush; Obiagwu, Chukwudi; Malhan, Namrita; Chawla, Kabu

doi:10.1177/2324709618757260

Cited by 4 publications

(4 citation statements)

References 15 publications

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“…There were few similar cases reported in the past 5 years. [5,[22][23][24][25][26][27][28] PAH is a rare and very severe AOSD manifestation, but the prevalence is probably under estimated because only symptomatic cases were reported. PAH may occur at AOSD onset or later course and seems to mainly affect women.…”

Section: Discussionmentioning

confidence: 99%

Macrophage activation syndrome and pulmonary arterial hypertension in a patient with adult-onset Still disease

Zeng¹,

Deng

Luo³

et al. 2019

Medicine

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Introduction: Macrophage activation syndrome (MAS) and pulmonary arterial hypertension (PAH) are rare and life-threatening complications of adult-onset Still disease (AOSD).Patient concerns: We reported an interesting case of a 25-year-old AOSD patient with MAS and PAH, and the patient was found to have right anomalous pulmonary venous connection accompanied by an atrial septal defect.Diagnosis: MAS was diagnosed as a complication of AOSD. PAH was contributed probably by right anomalous pulmonary venous connection. Interventions:The patient dramatically improved with methylprednisolone (80 mg I.V. daily) plus supportive treatments, without interleukin (IL) inhibitors or ciclosporin A given.Outcomes: The patient's serum hepatic enzyme levels dropped and hemocytes rose within 1 week. Conclusion:Other causes need to be excluded carefully before giving a diagnosis of PAH with AOSD. Early diagnosis and aggressive treatments are pivotal to improve the quality of life and the survival of patients. Abbreviations: ALT = alanine transaminase, AOSD = adult-onset Still disease, AST = aspartate transaminase, CRP = C-reactive protein, CTA = computed tomography angiography, CTDs = connective tissue diseases, ESR = erythrocyte sedimentation rate, Hb = hemoglobin, IL = interleukin, INF = interferon, LDH = lactate dehydrogenase, MAS = macrophage activation syndrome, NK = natural killer, PAH = pulmonary arterial hypertension, RBC = red blood cell, TG = triglyceride, TNF = tumor necrosis factor, WBC = white blood cell.

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Section: Discussionmentioning

confidence: 99%

Macrophage activation syndrome and pulmonary arterial hypertension in a patient with adult-onset Still disease

Zeng¹,

Deng

Luo³

et al. 2019

Medicine

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“…The recommended posology for patients with Still’s disease (AOSD and SoJIA) with body weight ≥7.5 kg is 4 mg/kg (up to a maximum of 300 mg) administered every 4 weeks via subcutaneous injection ( European Medicines Agency [EMA], 2017 ). Up to now, the use of Canakinumab has been reported in 24 AOSD patients ( Kontzias and Efthimiou, 2012 ; Banse et al, 2013 ; Eriksson et al, 2013 ; Barsotti et al, 2014 ; Lo Gullo et al, 2014 ; Maria et al, 2014 ; Rossi-Semerano et al, 2015 ; Colafrancesco et al, 2017 ; Sinha et al, 2018 ) and 29 older adolescents/young adults representing AOSD patients ( Feist et al, 2018 ), refractory to NSAIDs, DMARDs and often to other biologic therapy. The majority of patients showed a rapid and sustained efficacy with subcutaneous injection of 150 mg every 8 weeks.…”

Section: Therapymentioning

confidence: 99%

“…In the listed reports ( Table 1 ), Canakinumab was effective in the most difficult-to-treat cases of refractory AOSD, except in two patients ( Rossi-Semerano et al, 2015 ; Sinha et al, 2018 ). The former was a refractory AOSD patient with a rare cardiopulmonary manifestation (severe pulmonary hypertension), the latter was a refractory AOSD patient receiving 150 mg/4 week Canakinumab.…”

Section: Therapymentioning

confidence: 99%

Development and Role in Therapy of Canakinumab in Adult-Onset Still’s Disease

et al. 2018

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Adult-onset Still’s disease (AOSD) is a rare inflammatory disease of unknown etiology typically characterized by episodes of spiking fever, evanescent rash, arthralgia, leukocytosis, and hyperferritinemia. The pivotal role of interleukin (IL)-1 and other pro-inflammatory cytokines gives rise to the development of new targeted therapies. Currently, AOSD patients can benefit from efficient and well tolerated biologic agents, such as IL-1, IL-6, and tumour necrosis factor (TNF)-α antagonists. Canakinumab, a human monoclonal anti-IL-1β antibody, is indicated for the treatment of different autoinflammatory syndromes in adults, adolescents, and children and it has recently been approved for AOSD treatment. In this article, we summarize the structural and biochemical data describing the molecular interactions between Canakinumab and its target antigen. Some special considerations of the pharmacological properties of Canakinumab are included. We also review the safety, efficacy and tolerability of this drug for the treatment of AOSD.

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“…При БСВ, рефрактерной к предшествующей стандартной терапии НПВП, ГК, а также ингибиторами ФНОα, ИЛ6 и даже ИЛ1 (анакинра), опыт применения канакинумаба в целом весьма позитивен[45][46][47][48][49][50][51][52][53][54][55][56] (табл. 2).…”

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Therapy with canakinumab for adult-onset still's disease

Насонов

2019

Naučno-praktičeskaâ revmatologiâ

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Still's disease in children (systemic-onset juvenile idiopathic arthritis, SoJIA) and in adults (adult-onset Still's disease) are considered as non-familial systemic autoinflammatory diseases of unknown etiology driven by similar immunopathogenetic mechanisms. The adult-onset Still's disease pathogenesis is based on genetically determined innate immunity disturbances and molecular basis of immunopathogenesis consists of NLRP3 inflammasomedependent mechanisms of inflammation characterized by hyperproduction of proinflammatory cytokines interleukin (IL) 1 and IL18. Nonsteroidal anti-inflammatory drugs, glucocorticoids, methotrexate and other disease modifying drugs are considered as «first line» medications for the treatment of adult-onset Still's disease and if they fail biologicals are recommended. A review of the literature data concerning anti-IL1 monoclonal antibodies administration in adult-onset Still's disease is presented, indicating good prospects for the use of canakinumab not only in case of resistance to standard therapy, but also as a «first-line» therapy in the onset of the disease.

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Severe Pulmonary Hypertension Due to Adult-Onset Still’s Disease

Cited by 4 publications

References 15 publications

Macrophage activation syndrome and pulmonary arterial hypertension in a patient with adult-onset Still disease

Macrophage activation syndrome and pulmonary arterial hypertension in a patient with adult-onset Still disease

Development and Role in Therapy of Canakinumab in Adult-Onset Still’s Disease

Therapy with canakinumab for adult-onset still's disease

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