Pulmonary hypertension in a patient with adult-onset stills disease

Mubashir, E.; Ahmed, M. M.; Hayat, Samina; Heldmann, Maureen; Berney, S.M.

doi:10.1007/s10067-006-0382-3

Cited by 28 publications

(19 citation statements)

References 12 publications

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“…Although isolated case reports of PAH, AP, and ILD in systemic JIA and adult-onset Still's disease have been pub-lished (22)(23)(24)(25)(26)(27)29,30), the findings in this study indicate that these complications may occur more commonly than previously suspected and are fatal in the majority of patients. PAH, ILD, and AP are all processes in which inflammation likely has an important role; for example, pathologic PAH specimens show an increased inflammatory perivascular infiltrate, including macrophages, dendritic cells, lymphocytes, and mast cells (31)(32)(33).…”

Section: Discussionmentioning

confidence: 50%

“…Although pleuritis, along with pericarditis, is a commonly recognized feature of systemic JIA (11,21), other pulmonary complications, such as PAH, interstitial lung disease (ILD), and alveolar proteinosis (AP) or lipoid pneumonia, are extremely rare. Prior to 2008, only scattered single case reports of PAH, AP, ILD, and illnesses that may represent one of these conditions in systemic JIA and Still's disease existed in the literature (22)(23)(24)(25)(26)(27), so the number of cases being reported to the Listserv represented an increase. Because IL-1 inhibitors became much more commonly used in systemic JIA in the mid-2000s, there was concern about a possible association between exposure to biologic medications such as IL-1 inhibitors and the development of pulmonary complications in some patients (16,17).…”

Section: Introductionmentioning

confidence: 99%

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Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis

Kimura

Weiss

Haroldson

et al. 2013

Arthritis Care & Research

146

169

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Objectives Systemic Juvenile Idiopathic Arthritis (sJIA) is characterized by fevers, rash and arthritis, for which IL1 and IL6 inhibitors appear effective. Pulmonary artery hypertension (PAH), interstitial lung disease (ILD) and alveolar proteinosis (AP) have been recently reported in sJIA patients with increased frequency. Our aim was to characterize and compare these cases to a larger cohort of sJIA patients. Methods sJIA patients who developed PAH, ILD and/or AP were identified through an electronic listserv, and their demographic, sJIA and pulmonary disease characteristics, and medication exposure information were collected. These features were compared to a cohort of sJIA patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. Results Patients (N=25) were significantly (p<0.05) more likely than the CARRA registry cohort (N=389) to be female, have more systemic features, and to have been exposed to an IL-1 inhibitor, tocilizumab, infliximab, corticosteroids, intravenous immunoglobulin, cyclosporine and cyclophosphamide. Eighty% were diagnosed after 2004. Twenty (80%) patients had MAS during their disease course and 15 (60%) had MAS at pulmonary diagnosis. Sixteen patients had PAH, 5 AP and 7 ILD. Seventeen (68%) patients were taking or recently (≤1 month) discontinued a biologic agent at pulmonary symptom onset; 12 (48%) were taking anti-IL1 therapy (primarily anakinra). Seventeen (68%) patients died at a mean of 8.8 months from pulmonary diagnosis. Conclusions PAH, AP and ILD are under-recognized complications of sJIA which are frequently fatal. These may be the result of severe uncontrolled systemic disease activity, and may be influenced by medication exposure.

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Section: Discussionmentioning

confidence: 50%

Section: Introductionmentioning

confidence: 99%

Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis

Kimura

Weiss

Haroldson

et al. 2013

Arthritis Care & Research

146

169

View full text Add to dashboard Cite

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“…A retrospective analysis of 19 patients with PAH and various autoimmune diseases was done in northern Taiwan, where PAH has been reported seldom, and revealed that 2 patients had AOSD; in this study, 5 patients died and the RVSP correlated with the levels of serum uric acid ( r = 0.686, P = 0.001) [12]. Another case report described a 29-year-old woman, who 9 years after a diagnosis of AOSD, developed PAH (mean PAP 33 mmHg) responsive to vasodilator infusion, but died 2.5 months later of right heart failure, despite initiation of anakinra [13]. Lastly, a case report from Hyderabad, India, described an 18-year-old female with new onset AOSD who developed severe PAH (mean PAP of 65 mmHg); in the absence of other causes, the authors concluded that the PAH was secondary to the AOSD-related pulmonary microangiopathy [14].…”

Section: Discussionmentioning

confidence: 77%

Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra

Campos

Schiopu

2012

Case Reports in Rheumatology

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Adult-onset Still's disease (AOSD) is a rare inflammatory condition characterized by spiking quotidian fever, rash, chronic arthralgia, leukocytosis, and occasional pulmonary involvement such as pleural effusion and transient pulmonary infiltrates. Pulmonary arterial hypertension (PAH) is a rare pulmonary complication of AOSD, and we are aware of only 5 cases reported in the literature. We report the case of a 27-year-old woman of Middle Eastern descent, with a 7-year history of AOSD, who developed severe pulmonary arterial hypertension (PAH). After unsuccessful exposure to various immunosuppressive regimens, shortly following the initiation of anakinra, an interleukin-1 (IL-1) receptor antagonist, her disease became quiescent and the PAH resolved. With this case report, we hope to show that anakinra, either by virtue of controlling the overall inflammation in AOSD, or by direct effect on the pulmonary microangiopathy, can improve severe PAH.

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“…Therefore, the diagnosis of AOSD is usually based on clinical findings after exclusion of other diseases. AOSD usually has a good prognosis, but can be life-threatening, [5] especially when it is complicated with multiple organ failure and DIC caused by SIRS, [6–10] hemophagocytic syndrome, [11–13] thrombotic thrombocytopenic purpura, [5] acute respiratory distress syndrome, [14] diffuse alveolar hemorrhage, [15] pulmonary arterial hypertension, [16] and liver failure. [17] Therefore, these severe complications should not be overlooked when we see patients with AOSD.…”

Section: Discussionmentioning

confidence: 99%

Tocilizumab for uncontrollable systemic inflammatory response syndrome complicating adult-onset Still disease

Masui-Ito

Okamoto²,

Ikejiri³

et al. 2017

Medicine

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Rationale:Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, fever spikes, arthralgia, and lymphadenopathy. AOSD usually has a good prognosis, but it can sometimes be fatal, especially when it is complicated by systemic inflammatory response syndrome (SIRS) and multiple organ failure.Patient concerns:A previously healthy 26-year-old woman was referred to our hospital for persistent high fever and mild systemic edema. Five days later, the patient presented with dyspnea, hypotension, and anuria. Anasarca developed with massive pleural effusion, ascites, and systemic edema, resulting in an increase of 47 kg in body weight.Diagnoses:The patient was diagnosed as AOSD after infection, malignancy, hematologic disorders, and other autoimmune diseases were excluded.Interventions:We administered tocilizumab, an IL-6 receptor inhibitor, intravenously in addition to cyclosporine, prednisolone, plasma exchange, and continuous hemodiafiltration.Outcomes:The patient's systemic condition improved. After stabilization by all medications, the patient was managed and responded to tocilizumab alone. To the best of our knowledge, this was the first case of severe SIRS complicating AOSD that was successfully treated with an anti- IL-6 receptor antibody.Lessons:SIRS should not be overlooked in a patient with steroid-resistant AOSD and edema. Inhibitors of the IL-6 receptor can be used safely and effectively to control AOSD complicated with severe SIRS.

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Pulmonary hypertension in a patient with adult-onset stills disease

Cited by 28 publications

References 12 publications

Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis

Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis

Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra

Tocilizumab for uncontrollable systemic inflammatory response syndrome complicating adult-onset Still disease

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