Adult-onset Still's Disease With Pulmonary Involvement

Hijikata, Naoya; Takayanagi, Noboru; Sugita, Yutaka; Kawabata, Yoshinori

doi:10.1097/lbr.0b013e3181bdab74

Cited by 7 publications

(5 citation statements)

References 9 publications

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“…Non-specific histological findings are suggestive of dermal inflammation by lymphocytes and histiocytes with perivascular invasion. 18 An overwhelming majority of patients (64-100%) are distressed with arthritis and arthralgias affecting knees, ankles, and wrists as commonly targeted joints, but arthritis and arthralgias of elbow, shoulder, and hips are also reported. 19 Wrist manifestations depict the narrowing of joint space and ankylosis within 3 years of disease occurrence in untreated patients.…”

Section: Discussionmentioning

confidence: 99%

“…A case of AOSD with organising pneumonia based on HRCT and transbronchial biopsy findings has also been reported. 18 Proinflammatory cytokines, specifically IL-18, play a vital role in displaying pulmonary manifestations in AOSD. Acute intervals of the disease are characterised by peaking levels of IL-18 in serum and pulmonary tissues along with spiking levels of serum ferritin.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Interstitial Lung Disease: A Rare Association with Adult-Onset Still’s Disease

Asghar¹,

Fatmi²,

Zafar³

et al. 2020

EMJ Respir

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Pulmonary lung involvement in adult-onset Still’s disease (AOSD) can be classified into two categories: with or without acute respiratory distress syndrome. Interstitial fibrosis in AOSD is rare, occurring in less than 5% of cases. Here, the authors present a case of a 40-year-old male of Asian descent with a past history of hyperthyroidism who presented with fever, shortness of breath associated with cough, sore throat, diffuse arthralgias, pink-coloured rash, and hepatomegaly. Laboratory investigations revealed leukocytosis, abnormal liver function tests, negative antinuclear antibodies, and negative rheumatoid factor. Chest X-ray showed bilateral basal infiltrates, while high-resolution CT chest scan confirmed the diagnosis of interstitial lung disease in association with ASOD. This case suggested a direct association of AOSD and interstitial lung disease, but autoimmune pathogenesis is the only link synchronising both diseases and the exact mechanism of direct involvement is ambiguous.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Interstitial Lung Disease: A Rare Association with Adult-Onset Still’s Disease

Asghar¹,

Fatmi²,

Zafar³

et al. 2020

EMJ Respir

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“…Previous reports of patients with chronic persistent interstitial pneumonia detected by TBLB, indicate lymphocyte infiltration and mild fibrosis of the alveolar wall (7-9). Recently, Hijikata et al reported a case of AOSD in which TBLB revealed a pattern of organizing pneumonia (10). However, cases of AOSD presenting pulmonary manifestation detected by TBLB or BAL are quite rare.…”

Section: Discussionmentioning

confidence: 99%

A Case of Adult Onset Still's Disease Complicated with Cryptogenic Organizing Pneumonia

et al. 2011

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Only a few pathologic reports exist describing adult onset Still's disease (AOSD) with pulmonary involvement. We report this very rare case of AOSD complicated with cryptogenic organizing pneumonia (COP). A 32-year-old woman was referred with high spiking fever, salmon-pink rash in her arms and legs, and polyarthralgia. The laboratory data showed marked increases in white blood cell count, an erythrocyte sedimentation rate, and C reactive protein, ferritin, and liver dysfunction. All cultures remained negative, as were autoantibodies and rheumatoid factor. The patient was strongly suspected of AOSD according to specific diagnostic criteria. However, chest X ray disclosed an infiltrative shadow accompanied by air bronchogram in the upper lobe of the right lung and therapy with antibiotics was initiated. As the patient did not respond to antibiotics and a remittent fever of over 38 , a flexible bronchoscopy was performed. Organizing pneumonia was diagnosed by transbronchial lung biopsy (TBLB) histology and radiologically, and the lesions were thought to be due to pulmonary involvement of AOSD. Therefore, she was diagnosed with AOSD complicated with COP. Oral treatment with prednisolone (30 mg/day) resulted in rapid disappearance of the infiltrative shadow. Symptoms and markers of inflammation also improved. Clinicians should be aware that COP can be a complication of AOSD.

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“…Microscopic findings can yield a lymphoplasmacytic, nongranulomatous immune reaction as well as signs of intraalveolar (acute fibrinous) organizing pneumonia, bronchiolitis, and fibrotic remodeling (7,(49)(50)(51).…”

Section: Diagnosticsmentioning

confidence: 99%

Rare, rarer, lung involvement in adult-onset Still's disease: A mini-review

Nies¹,

Schneider

Krusche

2022

Front. Med.

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Adult-onset Still's disease (AOSD) is a polygenic systemic autoinflammatory disease which is associated with increased morbidity and mortality. Pulmonary involvement is a rare, but serious complication of AOSD. As in AOSD, IL-1b, IL-18, and IL-6 dominate the molecular pathogenesis, which mediate a type 1 and type 3 inflammatory signature of the adaptive immune system. This is evidenced by the success of IL-1- and IL-6 inhibition in the management of AOSD. However, anaphylactic reactions to treatment with IL-1- or IL-6-inhibitors is currently being discussed as a potential trigger for lung involvement inf AOSD, while genetic risk factors have also been identified. Clinically, pulmonary involvement in AOSD can manifest in many different forms. Parenchymal inflammation with peripheral consolidations is the most frequent form while PAH is less common, but often very difficult to manage. This mini-review provides an overview of the pathophysiology as well as the clinical presentation and the diagnostic features of pulmonary involvement in AOSD.

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Adult-onset Still's Disease With Pulmonary Involvement

Abstract: AOSD can produce lung involvement. TBLB is useful in the diagnosis of pulmonary lesions.

Cited by 7 publications

References 9 publications

Interstitial Lung Disease: A Rare Association with Adult-Onset Still’s Disease

Interstitial Lung Disease: A Rare Association with Adult-Onset Still’s Disease

A Case of Adult Onset Still's Disease Complicated with Cryptogenic Organizing Pneumonia

Rare, rarer, lung involvement in adult-onset Still's disease: A mini-review

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