A Case of Bronchiolitis Obliterans Organizing Pneumonia Associated with Primary Sjögren’s Syndrome Who Died of Superimposed Diffuse Alveolar Damage

Usui, Yukio; Kimula, Yuji; Miura, Hiroshi; Kodaira, Y.; Takayama, Shigemitsu; Nakayama, Masayuki; Kataoka, N.

doi:10.1159/000196041

Cited by 30 publications

(18 citation statements)

References 6 publications

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“…In addition, the association between LIP and PSS has also been reported by other investigators [23,24,25]. Furthermore, it has been described that OP [26,27,28], usual interstitial pneumonia (UIP) and NSIP [29,30] can also be associated with PSS.…”

Section: Discussionsupporting

confidence: 65%

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Pulmonary Manifestations of Sjögren’s Syndrome

Shi

Liu²,

et al. 2009

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Background: Primary Sjögren’s syndrome (PSS) is associated with various histological patterns of interstitial lung disease. Although chest images and lung function studies showed that lung involvement predominantly occurs in small airways, pathological findings were not consistent with the results of high-resolution CT (HRCT) and lung function tests. Objectives: To investigate the pathological characteristics of PSS-associated interstitial lung disease (PSS-ILD) and their relationship with HRCT lung function tests. Methods: Fourteen patients diagnosed as PSS who underwent surgical lung biopsy in Peking Union Medical College Hospital from October 2000 to October 2006 were reviewed. Histopathologic findings, radiologic findings and lung function tests were analyzed. Results:The study included 13 women. The median age was 46 years. Most patients presented with dyspnea and cough. CT scans revealed bilateral ground-glass, consolidative, reticular and nodular opacities and cyst lesions. The histological patterns included nonspecific interstitial pneumonia (NSIP) cellular pattern associated with organizing pneumonia (OP), NSIP mixed pattern associated with OP, noncaseating granulomas, chronic bronchiolitis, follicular bronchiolitis, constrictive bronchiolitis, lymphocytic interstitial pneumonia associated with follicular bronchiolitis, NSIP mixed pattern associated with follicular bronchiolitis, NSIP mixed pattern coexisting with chronic bronchiolitis, OP associated with chronic bronchiolitis, and noncaseating granulomas coexisting with OP. Treatment included prednisone and cyclophosphamide. During the follow-up period (median 38 months), most patients improved or remained stable. The patient with constrictive bronchiolitis died from progression of primary disease. Conclusions: The histopathologic patterns of PSS-ILD included lung interstitial involvement and small airway involvement or both. Corticosteroid therapy combined with cyclophosphamide was administered with a favorable response in the majority of patients.

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Section: Discussionsupporting

confidence: 65%

“…Surgical lung biopsies were employed in three articles [22,24,29], and TBLB was used in four studies [23,25,26,27]. Specimens were obtained by surgical lung biopsy or TBLB in one study [11], and the methods of obtaining lung biopsy were not mentioned in two articles [5,30].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Manifestations of Sjögren’s Syndrome

Shi

Liu²,

et al. 2009

Respiration

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“…Organising pneumonia could even reveal Sjögren's syndrome [109][110][111][112]. Clinical presentation is similar to other organising pneumonia aetiologies.…”

Section: Organising Pneumonitismentioning

confidence: 99%

Pulmonary manifestations of Sjögren's syndrome

et al. 2016

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In 9–20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD) and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients.

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“…The diagnosis is suggested if antibiotics prove ineffective in such cases of pneumonia. In most of these published cases, the OP revealed SS [70][71][72][73] and regressed with corticosteroid therapy without sequelae. Cystic lesions have been reported during the course of SS [74].…”

Section: Bronchoalveolar Lavage Findingsmentioning

confidence: 99%