Bronchiolitis obliterans organising pneumonia as an initial manifestation in a patient with systemic lupus erythematosus: a rare presentation.

Bronchiolitis obliterans organising pneumonia as an initial manifestation of systemic lupus erythematosus (SLE) is a rare and uncommon presentation. We describe a case of SLE presenting with shortness of breath, found to have pneumothorax, bilateral nodular infiltrates along with pleural effusions and pericardial effusion. Work-up suggested a diagnosis of active SLE with anaemia, thrombocytopenia, positive antinuclear antibodies (ANAs) and positive anti-double-stranded DNA. On retrospective review of patient records, from 8 years prior to presentation, lung biopsy histology consistent with bronchiolitis obliterans organising pneumonia with positive ANA serology was found, without any further autoimmune work-up. In our opinion, bronchiolitis obliterans organising pneumonia was the index presentation of SLE. Treatment with steroids and subsequent management with immunosuppressive therapy could have prevented subsequent hospitalisations. Prompt work-up for autoimmune diseases should be considered in patients with positive ANA and histological evidence of bronchiolitis obliterans organising pneumonia.

Surgery of a nocardia lung abscess presenting as a tension pyopneumothorax.

While some cases of nocardial pneumonia develop secondary empyema, tension pyopneumothorax is a very rare and lethal complication. A 74-year-old man who exhibited thrombocytopenia during steroid therapy for autoimmune hepatitis, presented to our department with a nocardial tension pyopneumothorax. He underwent a left lower lobectomy after chest drainage, and was discharged without any complication other than reoperation to remove a postoperative hematoma.

Effects of early and late pneumothorax drainage on the development of pulmonary oedema.

We analyzed the effects of pneumothorax duration and early or late drainage on lung histology and biological markers associated with inflammation, alveolar fluid clearance, and pulmonary oedema formation. Pneumothorax was induced by injecting air into the thorax of anaesthetized rats, which were randomized according to duration of pneumothorax [5 (PTX5) or 30 (PTX30)min] and further divided to be drained (D) or not (ND). ND rats were euthanized at 5 and 30min. In D groups, pneumothorax was drained and rats breathed spontaneously for 30min. PTX30-ND, compared to PTX5-ND, showed higher alveolar collapse and oedema, type III procollagen, caspase-3, epithelial sodium channel-α, and aquaporin (AQP)-1 mRNA expression, and epithelial and endothelial damage, with reduced cystic fibrosis transmembrane conductance regulator (CFTR) and AQP-3 expression. PTX5-D, compared to PTX30-D, showed less alveolar hyperinflation, oedema, and alveolar-capillary damage, with reduced interleukin-6, caspase-3, AQP-5, and Na,K-ATPase-α and -ß expression, and increased CFTR expression. In conclusion, longer duration pneumothorax exacerbated lung damage, oedema, and inflammation.

Innate type 2 immunity is associated with eosinophilic pleural effusion in primary spontaneous pneumothorax.

RATIONALE: Eosinophilic pleural effusion (EPE) is characterized by greater than 10% eosinophilia and is frequently associated with air and/or blood in the pleural cavity. Primary spontaneous pneumothorax (PSP), defined as the spontaneous presence of air in the pleural space, is one of the most common causes of EPE. Recent studies have shown that type 2 immune responses play important roles in eosinophilic airway inflammation resulting in pleural pathology. OBJECTIVES: To determine the predominant immune responses associated with PSP in humans, and to examine whether IL-33, thymic stromal lymphopoietin (TSLP), or type 2 innate lymphoid cell (ILC2)-mediated immune responses are associated factors. METHODS: Eosinophil-associated cytokines were measured in the pleural fluid of patients with PSP and control subjects. Th2 cell and ILC2 responses in the pleural cavity and peripheral blood were also evaluated by in vitro restimulation and intracellular cytokine staining of T cells and ILC2s in patients with PSP (n = 62) and control subjects (n = 33). IL-33-mediated IL-5 production by ILC2s was also evaluated. MEASUREMENTS AND MAIN RESULTS: Significantly higher concentrations of IL-5 and eotaxin-3 were detected in the pleural fluid of patients with PSP, in addition to significantly higher concentrations of IL-33 and TSLP. Although IL-5 production was induced by IL-33 treatment of ILC2s, other Th2 cell-mediated immune responses were not detected. CONCLUSIONS: Our results indicate that innate immune responses characterized by the production of IL-33, TSLP, and IL-5 are associated with the development of EPE in PSP by an ILC2-dependent and Th2-independent mechanism.

A unique, histopathologic lesion in a subset of patients with spontaneous pneumothorax.

CONTEXT: Spontaneous pneumothorax can be idiopathic (primary), or it can occur in association with an underlying predisposing condition (secondary). Spontaneous pneumothorax may be a harbinger of an undiagnosed clinical condition, which may be associated with serious systemic abnormalities, making early recognition and diagnosis important. The pulmonary pathology of some of these disorders has not been fully elucidated. OBJECTIVE: To review cases of pneumothorax in the hope of identifying pathologic features that might correlate to specific clinical syndromes. DESIGN: The pathology computer files at 3 hospitals were searched for all cases of spontaneous pneumothorax, primary and secondary, regardless of etiology during a 11-year period. Ninety-two cases were retrieved. Each of the cases was evaluated for reactive eosinophilic pleuritis, elastosis, pleural fibrosis, emphysema, intra-alveolar macrophages, cholesterol clefts, vasculopathy, and intraparenchymal or intrapleural cysts. Clinical information regarding asthma and smoking history, site of the pneumothorax, family history, radiographic findings, predisposing conditions, recurrence, age, and sex were extracted from the medical records. RESULTS: In 11 patients (12% of all the patients with spontaneous pneumothorax), a distinctive pattern of pleural fibrosis with islands of fibroblastic foci within a myxoid stroma was noted at the pleural-parenchymal interface or leading edge. These lesions correlated with a select subset of patients, consisting predominantly of young men. CONCLUSIONS: Our review identified a distinct pattern of pneumothorax-associated fibroblastic lesions in a subset of cases of spontaneous pneumothorax. Whether this is related to the pathogenesis of the pneumothorax remains to be elucidated.

[Immuno-histochemical study contribution in thoracic endometriosis: about an analysis of eight cases]. / Apport de l'étude immuno-histochimique dans les localisations intrathoraciques de l'endométriose : à propos de huit cas.

Thoracic endometriosis (TE) is rare with positive histological diagnosis sometimes difficult, particularly in atypical form. The aim of this study was to identify features which can increase performance of the histolological TE diagnosis and more particularly immuno-histochemical (IHC) contribution with hormonal receptors, smooth muscle actin, Ber-Ep4, CD10 and calretinin antibodies. To address this issue, we retrieved, retrospectively, a large series of 591 pneumothorax operated. Among them, 135 (23%) were females including eight (6%) cases related to TE. Those eight women were surgically treated with resection of pleura (n=6/8), lung (n=5/8) and diaphragmatic samples (n=6/8). Typical histological lesions of endometriosis were observed in six cases among eight. All diaphragmatic samples presented, macroscopically, holes responsible of thoraco-abdominal communication (n=6/6). Endometrial glands and/or endometrial stroma cells were found in the diaphragm (n=5/6) and in the pleura (n=2/6) but were never encountered in the lung (n=0/5). IHC study can confirm the five diaphragmatic localizations and can identify a new localization with expression of hormonal receptors, CD10 and smooth muscle actin in an island of fusiform cells. In conclusion, our study shows 1) the high frequency of diaphragmatic endometriosis localization which holes existence also can explain the pathogenesis, 2) the value of diaphragmatic samples in positive histological diagnosis of TE, 3) IHC interest to confirm endometriosis, particularly in atypical form and to differentiate from mesothelial cells inclusion.

Video-assisted thoracoscopic surgery for primary spontaneous pneumothorax: clinicopathological correlation.

OBJECTIVE: To compare the identifiable pulmonary abnormalities during thoracoscopy with the histological findings in patients requiring surgical intervention for recurrent or persistent primary spontaneous pneumothorax (PSP) and correlate these with the postoperative events. METHODS: From January 1999 to December 2002, 94 consecutive patients underwent video-assisted thoracoscopic wedge excision and apical pleurectomy for PSP. Vanderschueren's classification was used for macroscopic staging and histological observation for microscopic features. Clinical data of these patients and the outcome of surgery were described. RESULTS: All patients were successfully treated using video-assisted thoracoscopic technique. Recurrent pneumothorax was the most frequent indication for surgery, occurring in 60 cases. The method of management was stapling of an identified bleb or apex of the upper lobe and apical pleurectomy. In 67 cases (71%), clear bullae were found in types III and IV. In 15 cases (16%), type II pleuropulmonary adhesions were identified and in 12 (13%) cases thoracoscopy failed to reveal any abnormality (type I). The actual site of air leakage could be located during thoracoscopy in 24 (26%) patients. Histologically, 74 patients had subpleural bullae/blebs formation and 20 had emphysema without bullae. Fifty-three patients had cellular infiltration and 82 had pleural fibrosis. In the microscopic examination, the actual site of air leakage could be located at the site of subpleural blebs or bullae in 15 patients and elsewhere at the lung surface in five other patients. Postoperative prolonged air leak occurred in 4 out of 12 patients in type I and in two of the remaining patients, p=0.001. Mean follow-up is 48 months (range, 30-60 months) for all patients. Pneumothorax recurred in three patients (3.1%). Two patients from type I (16.6%) and one patient from the other types (1.2%) had recurrence (p=0.01). CONCLUSIONS: Video-assisted thoracoscopic stapling of an identified bleb or apex of the upper lobe and apical pleurectomy represents the standard treatment for the majority of recurrent or persistent PSP. Most patients with surgically treated PSP have subpleural blebs or bullae or isolated emphysema. In type I cases, simple apical excision and apical pleurectomy are not sufficient and perhaps additional talc poudrage might be indicated.

[Intracavitary laser therapy and its effect on the kallikrein-kinin system of blood in children with pneumothorax].

The article presents the results of low-intensity laser application in complex treatment of 137 children with acute purulent destructive pneumonia complicated by pneumothorax with bronchial fistulas. A method of intracavitary laser therapy, developed in the clinic, allowed obliteration of bronchopleural fistulas without application of bronchial occlusion and other invasive techniques. Evaluation of the kallikrein-kinin system of blood revealed prominent reduction of kininogenesis in most (87%) patients upon admission (3 weeks after the onset of the disease), which is an important link of the pathogenesis of late stages of complicated acute purulent lung destruction in children. The study also demonstrated that low-intensity laser emission modulates pyoinflammatory process due to its effect on cell-mediated immunity, neutrophilic phagocytosis and the kallikrein-kinin system of blood. Intracavitary laser therapy is the treatment of choice in children with acute purulent destructive pneumonia complicated by pneumothorax with bronchial fistulas. Application of intracavitary laser therapy in complex therapy of complicated acute purulent lung destruction in children allowed discharge from the hospital 5 to 7 day earlier, and prevented lung inflammatory process chronization. None of the patients have died within last 10 years.

Pneumothorax-associated pleural eosinophilia in mice is interleukin-5 but not interleukin-13 dependent.

STUDY OBJECTIVES: To establish a murine model of pneumothorax-associated pleural eosinophilia and to examine the role of interleukin (IL)-5 and IL-13 in the pathogenesis of this reaction. DESIGN: An animal study. INTERVENTIONS: One hundred thirty-seven C57/Bl-6 mice were used in this study. Wild-type animals were injected intrapleurally with 0.4 mL of air and were killed at different time points (30 min to 7 days) after the injection. IL-5 knockout and IL-13 knockout animals were killed 24 h and 48 h after the injection. Pleural inflammation was assessed by pleural lavage (PL). MEASUREMENTS AND RESULTS: PL cells were significantly increased following the induction of pneumothorax. The peak number of neutrophils, observed at 12 h, was 900 times higher than the control. The peak number of eosinophils, observed at 48 h, was 700 times higher than the control. Lymphocytes and mononuclear cells increased threefold and fourfold, respectively. IL-5 knockout mice had significantly less PL eosinophils than that the wild-type or the IL-13 knockout mice at 24 h (150 +/- 46/microL, 903 +/- 244/microL, and 912 +/- 168/microL, respectively; p = 0.013) and 48 h (181 +/- 45/microL, 1,587 +/- 212/microL, and 1,379 +/- 364/microL, respectively; p = 0.003). CONCLUSION: Pneumothorax induces an inflammatory reaction of the mouse pleura, mainly characterized by increased neutrophils and eosinophils. IL-5 but not IL-13 is required for pneumothorax-associated pleural eosinophilia.

[Catamenial pneumothorax long-term monitored by serum levels of CA 125; report of a case].

A 46-year-old woman was admitted to our hospital because of the repeating right chest pain. Chest X-ray showed a right pneumothorax. This symptom corresponded with her mensturations and elevated serum levels of CA 125. We diagnosed catamenial pneumothorax. Hormones therapy was not performed because severe side effects. For that reason we performed thoracoscopic surgery. We found many little sized fistulas on diaphragm and blue berry spots on right upper lobe during the operation. She didn't recurrence for 2 years after that operation.

Concurrent left-sided spontaneous pneumothorax in Japanese monogerminal twins.

Monogerminal twin brothers aged 17 were admitted because of concurrent left-sided spontaneous pneumothorax. A familial background of this common disease in association with the human leukocyte antigen (HLA) has been suggested; however, the actual mode of inheritance in association with HLA typing is still uncertain. HLA analysis in this family revealed the HLA-A24, B61 (40), Cw3, DR4, DR53, and DQ3 haplotype in the twins and their father, who also had a medical history of spontaneous pneumothorax. We report these cases as an extremely rare example of familial occurrence of spontaneous pneumothorax.

Basophils from patients with allergic asthma show a primed phenotype.

BACKGROUND: IL-3, IL-5, and GM-CSF are not able to induce histamine release in purified basophils of nonallergic donors. However, we have recently found that preincubation with 2 micromol/L thapsigargin, which induces a rise in intracellular free calcium ions, renders human basophils extremely sensitive for IL-3, IL-5, or GM-CSF, leading to enhanced histamine release. Histamine release was also induced in the reverse order (first cytokine and then thapsigargin). OBJECTIVE: Because these cytokines are supposed to be increased in allergic inflammation, we examined whether basophils of patients with allergic asthma showed an enhanced response to thapsigargin. METHODS: We measured the histamine release induced by thapsigargin in a group of allergic asthmatic subjects (n = 24) and compared this response with those of 3 control groups. The control groups consisted of healthy control subjects (group 1, n = 21); patients with a nonallergic, nonasthmatic lung disease (group 2, n = 22); and patients with nonallergic asthma (group 3, n = 9). RESULTS: There was no difference in spontaneous histamine release. Also, no significant difference in histamine release was found when anti-IgE or formyl-methionyl-leucyl-phenylalanine was used as a stimulus. Histamine release induced by IL-3 alone or a combination of IL-3 and thapsigargin also did not differ. In contrast, basophils from the group with allergic asthma showed a significantly higher percentage of histamine release induced by thapsigargin (38.2% +/- 13.2%) than did basophils from the 3 control groups (healthy control subjects, 22.5% +/- 6.9%; subjects with lung disease, 24.9% +/- 8.9%; subjects with nonallergic asthma 15.0% +/- 3.0%; all mean +/- SD). CONCLUSION: These data indicate that basophils in peripheral blood of subjects with allergic asthma have a primed phenotype and that thapsigargin-induced histamine release is a practical tool to study this phenomenon.

Expression of CA125 in thoracic endometriosis in a patient with catamenial pneumothorax.

A 40-year-old woman had experienced monthly right thoracic pain and productive cough occurring at the beginning of her menstrual period. X-ray findings indicated a diagnosis of catamenial pneumothorax. The serum CA125 level was very high at 159.6 U/ml. Thoracoscopy showed multiple dark cherry-colored nodules with neovascularization on the diaphragm. Following partial resection of the diaphragm thoracic endometriosis was diagnosed. Immunohistochemical staining of these endometrial cells showed antibodies to CA125. She has been well without recurrence for 15 months, and her serum CA125 level was within the normal range after operation.

AIDS-related Pneumocystis carinii pneumonia in the era of adjunctive steroids: implication of BAL neutrophilia.

Factors predictive of mortality in patients with AIDS and Pneumocystis carinii pneumonia (PCP) were identified before the introduction of adjunctive steroids, but they have not been reevaluated since. Because PCP still occurs in AIDS, remaining fatal in some cases, we conducted a multivariate analysis of factors predicting mortality in patients with HIV-positive PCP managed from 1990 to 1995, i.e., after the consensus conference on the use of adjunctive steroids. The predictive value of clinical, laboratory, and bronchoalveolar lavage (BAL) data at admission and during the course of PCP was studied retrospectively using multivariate methods, in 144 patients with AIDS. Overall mortality was 21.5%. The univariate analysis identified seven factors predictive of 90-d mortality: Pa(O(2)) on room air < 60 mm Hg, lactate dehydrogenase > 1,000 IU, albuminemia < 30 g/L, BAL neutrophilia > 10%, nosocomial infection, pneumothorax, and a need for mechanical ventilation. Four of these factors were independently associated with 90-d mortality in the multivariate analysis; among them, two were evaluable at admission, namely, Pa(O(2)) < 60 mm Hg on room air and BAL neutrophilia > 10%, and two during hospitalization, namely, the development of pneumothorax and a need for mechanical ventilation. Moreover, BAL neutrophilia was correlated to occurrence of pneumothorax and a need for mechanical ventilation. In the era of adjunctive steroid use, AIDS-related PCP remains fairly common. Two independent factors evaluable at admission, Pa(O(2)) on room air and BAL neutrophilia, are predictive of death.

[A case of catamenial pneumothorax with elevated level of serum CA 125].

A 40-year old woman had experienced repeated spontaneous pneumothorax on the right side at the beginning of each menstrual period for one year. The level of serum CA125 was high at 60 ng/ml. Right thoracotomy revealed the presence of multiple darkly-pigmented spots and several pinhole like perforations near the central tendon of the diaphragm. The partial resection of the diaphragm was done, endometrial tissues were detected histologically. Tha patient has been asymptomatic after operation and the level of serum CA125 is normal.

Complement anaphylatoxin C3a and C5a formation in premature children with respiratory distress.

UNLABELLED: Premature children (n = 25) with respiratory distress (RD) were studied regarding complement activation and formation of the anaphylatoxins C3a and C5a. Blood samples were drawn on admission to the paediatric intensive care unit. In 18 of the patients RD was accompanied by other perinatal complications like pneumothorax or intracerebral haemorrhages. Seven of the premature children had RD without such complications. Preterm children with RD and with peri- and postnatal complications such as pneumothorax or intracerebral haemorrhage had increased concentrations in plasma of the anaphylatoxins C3a and C5a compared with preterm children with RD without these complications. There was a positive correlation between the plasma C3a and C5a concentrations in the preterm children. CONCLUSION: The present study indicates that isolated RD will appear without signs of complement activation and that complications like pneumothorax or intracerebral haemorrhages are associated with release of the anaphylatoxins C3a and C5a.

Quantitative analysis of carcinoembryonic antigen, squamous cell carcinoma antigen, CA 125, and CA 50 cytosolic content in non-small cell lung cancer.

BACKGROUND: The cytosolic content of carcinoembryonic antigen (CEA), squamous cell carcinoma (SCC), CA 125, and CA 50 antigens in non-small cell lung cancer (NSCLC) is analyzed in this study. The aim was to ascertain the relationship between tumor marker content and the clinicopathologic aspects of this neoplasm. METHODS: Lung tissue samples were obtained at the time of surgery from 75 patients with NSCLC patients (samples of tumor and unaffected tissue) and 29 subjects with idiopathic pneumothorax. All determinations were performed on cytosols obtained from lung specimens. CEA and CA 125 were determined by enzyme immunoassay, SCC antigen by radioimmunoassay, and CA 50 by fluoroimmunoassay. Tumor marker content was analyzed by TNM stage, histologic type, tumor grade, and number of atypias. RESULTS: The concentration of the four markers was significantly higher in cytosol obtained from neoplastic tissue. Frequency of elevated levels of CEA was higher in adenocarcinoma (87% cases expressing high levels of the marker), SCC antigen in epidermoid carcinoma (65% expressing high levels), and CA 125 in large cell carcinomas (100% expressing high levels). No association was found between TNM stage and cytosol concentration for any of the four markers. CEA exhibited significantly greater concentration in well differentiated tumors, whereas this was true of CA 125 in poorly differentiated tumors. CA 125 content was higher in tumors with more atypia. CONCLUSIONS: Cytosolic quantification of tumor markers may be an adjuvant mechanism to evaluate histologic subtypes of non-small cell lung cancer and identification of tumors with poorly differentiated features.

Reactive eosinophilic pulmonary vascular infiltration in patients with spontaneous pneumothorax.

Prominent nonnecrotizing eosinophilic inflammation of muscular pulmonary arteries was seen in resected lung tissue from two patients with spontaneous pneumothorax. Other histologic features included reactive eosinophilic pleuritis (REP) and fibrobullous disease. Eosinophilic vascular infiltration was not contiguous to REP. In neither patient was there a specific and recognized cause of eosinophilic vasculitis. Both patients are without pulmonary symptoms 1 and 4 years after pneumothorax. Eosinophilic vascular infiltration initially suggested the diagnosis of allergic angiitis or pulmonary eosinophilic granuloma. These diagnoses were excluded by clinical and morphologic data. We subsequently reviewed 30 cases of lung tissue resected from patients with pneumothorax and found REP in 18 patients (60%) and mild pulmonary vascular and perivascular eosinophilia in five patients (17%). REP was present in all cases with eosinophilic vascular infiltration. We conclude that this eosinophilic vascular lesion is an unusual reaction in patients with REP and pneumothorax. Occasionally this lesion mimics allergic angiitis or eosinophilic granuloma. The pathogenesis is probably related to vascular transport of eosinophils to the injured pleural surface.

[Studies on HLA typing in a family line with spontaneous pneumothorax].

Our patient with spontaneous pneumothorax is the oldest brother of 3 siblings. Pneumothorax had occurred in his younger sister and in 3 of his mother's 4 siblings also. Namely, pneumothorax had occurred in 5 (including our patient) of 7 persons, family line on mother's side, and thoracotomy had been performed in 3 of them. Two cases in which operative findings were obtained were introduced. Next, HLA and alpha 1-antitrypsin (alpha 1-AT) were determined in this family line. As the result, the alpha 1-AT all remained in the normal range and HLA typing was not in a special relation to the occurrence of pneumothorax in this family line.