Pulmonary capillary hemangiomatosis diagnosed by pathology of explanted lungs: a unique etiology serves as a key of clinical diagnosis

Wada, Hironobu; Nakajima, Takahiro; Suzuki, Hidemi; Anazawa, Rie; Narita, Tomoharu; Terada, Jiro; Yoshida, Shigetoshi; Nakatani, Yukio; Yoshino, Ichiro

doi:10.1007/s11748-018-0950-x

Cited by 6 publications

(6 citation statements)

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“…Montani et al [10] have reported that occupational exposure to organic solvents might present a risk factor for PVOD. Although crucial causes of risk factors of PCH have not been identified yet, PCH can be developed secondarily in lungs with PVOD, connective tissue diseases, and chronic passive congestion based on a few case reports [19]. Considering the above relation between PCH and PVOD, occupational exposure to silica or organic solvent in the current patient might have influenced the clinical course of PCH, although whether and to what extent of the exposure might be related to the development of PCH are unclear.…”

Section: Discussionmentioning

confidence: 99%

A case of early diagnosis of pulmonary capillary hemangiomatosis in a worker with exposure to silica

et al. 2019

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Background Pulmonary capillary hemangiomatosis (PCH) is a progressive and refractory vascular disease in the lung. Pulmonary hypertension is frequently combined with PCH when capillary proliferation invades to nearby pulmonary vascular systems. It is difficult to differentiate PCH from other diseases such as pulmonary venoocclusive disease and pulmonary arterial hypertension that cause pulmonary hypertension as they frequently overlap. Case presentation A 29-year-old female who had worked at a bathtub factory presented with progressive exertional dyspnea for the past 2 years. Computed tomography revealed centrilobular, diffusely spreading ground-glass opacities sparing subpleural parenchyma with some cystic lesions and air-trapping in both lungs, suggesting a peculiar pattern of interstitial lung disease with airway involvement. There was not any evidence of right heart failure or pulmonary hypertension on echocardiogram, as well as radiography. Microscopic examination of the lung by thoracoscopic resection showed atypical proliferation of capillary channels within alveolar walls and interlobar septa, without invasion of large vessels. Conclusion We experienced a pathologically diagnosed PCH in a young female complaining progressive dyspnea with prior exposure to occupational silica or organic solvent without elevated right ventricular systolic pressure (RVSP) who showed atypical pattern of radiologic findings.

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Section: Discussionmentioning

confidence: 99%

A case of early diagnosis of pulmonary capillary hemangiomatosis in a worker with exposure to silica

et al. 2019

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“…Typical pattern of CT in PCH and PVOD. (a) Unique pattern of PCH: GGOs distributed diffusely in a bat-wing pattern in the lung field, sparing subpleural parenchyma, 10 (b) typical pattern of PCH: diffuse centrilobular GGOs with poorly defined nodular pattern, 13 and (c) typical pattern of PVOD: centrilobular GGOs with poorly defined nodular pattern, septal lines, and mediastinal lymph node enlargement (white dotted lines). 6 enlargement were observed less frequently (19% and 12%, respectively) than in PVOD.…”

Section: Discussionmentioning

confidence: 99%

“…Among these cases, only two cases reported the findings of BAL. 10,11 Conversely, no cases reported transbronchial lung biopsy (TBLB). However, ''lung biopsy'' (not including open lung biopsy) was performed in four cases ( Table 2).…”

Section: Physiological Findings (Pfts and Bal)mentioning

confidence: 99%

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Features of radiological and physiological findings in pulmonary capillary hemangiomatosis: an updated pooled analysis of confirmed diagnostic cases

et al. 2019

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Pulmonary capillary hemangiomatosis (PCH) is a very rare and refractory disease characterized by capillary angioproliferation. The updated classification of pulmonary hypertension categorizes PCH into a subgroup of pulmonary arterial hypertension (PAH) alongside pulmonary veno-occlusive disease (PVOD). However, the definitive diagnosis of PCH only with noninvasive tools remains difficult. The aim of this study was to elucidate the radiological and physiological characteristics of PCH. We searched for cases of pathologically confirmed PCH in the English literature published between 2000 and 2018. We identified 26 cases among 39 studies. Then, we extracted and evaluated the relevant clinical information in all cases with available data. On chest computed tomography (CT), ground-glass opacities (GGOs) were observed in 92% of the cases, in which poorly defined nodular pattern was the most common (88%). GGOs in a bat-wing distribution were observed in one case. Septal lines and lymph node enlargement were observed less frequently (each 19%, 12%). Seven cases (27%) had overlapping abnormalities. Diffusing capacity of the lung for carbon monoxide (DLCO) was remarkably decreased. Alveolar hemorrhage by histological findings or bronchoalveolar lavage (BAL) was observed in seven cases. The present study showed that the most characteristic findings of CT in PCH was centrilobular GGOs with a poorly defined nodular pattern, and septal lines and lymph node enlargement were seen less frequently. Alveolar hemorrhage detected by BAL and decreased DLCO may also be helpful to recognize the possibility of PCH like PVOD.

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“…Nomenclature and terminology continue to be problematic in the field of lung alveolarization and BPD. A recent plea was made for the proper pluralization of "septum" as "septa" (402), and yet, the continued use of the incorrect "septae" persists in the "lung literature," (56,226,234,347,350,422,424,551,607). The pluralization of "alveolus" has also seen the recent introduction of a new misnomer: "alveolae" (594) instead of the correct form, "alveoli.…”

Section: Nomenclaturementioning

confidence: 99%

Recent advances in our understanding of the mechanisms of lung alveolarization and bronchopulmonary dysplasia

Lignelli

Palumbo

Myti

et al. 2019

American Journal of Physiology-Lung Cellular and Molecular Phys

111

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Bronchopulmonary dysplasia (BPD) is the most common cause of morbidity and mortality in preterm infants. A key histopathological feature of BPD is stunted late lung development, where the process of alveolarization—the generation of alveolar gas exchange units—is impeded, through mechanisms that remain largely unclear. As such, there is interest in the clarification both of the pathomechanisms at play in affected lungs, and the mechanisms of de novo alveoli generation in healthy, developing lungs. A better understanding of normal and pathological alveolarization might reveal opportunities for improved medical management of affected infants. Furthermore, disturbances to the alveolar architecture are a key histopathological feature of several adult chronic lung diseases, including emphysema and fibrosis, and it is envisaged that knowledge about the mechanisms of alveologenesis might facilitate regeneration of healthy lung parenchyma in affected patients. To this end, recent efforts have interrogated clinical data, developed new—and refined existing—in vivo and in vitro models of BPD, have applied new microscopic and radiographic approaches, and have developed advanced cell-culture approaches, including organoid generation. Advances have also been made in the development of other methodologies, including single-cell analysis, metabolomics, lipidomics, and proteomics, as well as the generation and use of complex mouse genetics tools. The objective of this review is to present advances made in our understanding of the mechanisms of lung alveolarization and BPD over the period 1 January 2017–30 June 2019, a period that spans the 50th anniversary of the original clinical description of BPD in preterm infants.

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Pulmonary capillary hemangiomatosis diagnosed by pathology of explanted lungs: a unique etiology serves as a key of clinical diagnosis

Cited by 6 publications

References 9 publications

A case of early diagnosis of pulmonary capillary hemangiomatosis in a worker with exposure to silica

A case of early diagnosis of pulmonary capillary hemangiomatosis in a worker with exposure to silica

Features of radiological and physiological findings in pulmonary capillary hemangiomatosis: an updated pooled analysis of confirmed diagnostic cases

Recent advances in our understanding of the mechanisms of lung alveolarization and bronchopulmonary dysplasia

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