Dieulafoy's disease of the bronchus: rare but potentially fatal: a case report and a review of literature.

BACKGROUND: Dieulafoy's disease of the bronchus can cause massive and even fatal hemoptysis. Even though it is rare, it should be considered by physicians all over the world. This paper reports a case of bronchial Dieulafoy's disease and summarizes the data of similar cases reported in literature. METHODS: We report a case of bronchial Dieulafoy's disease (BDD) in Tunisia. We also present a review of literature related to BDD from 1995 to 2022 using the PubMed, Google Scholar, web of science and Chinese National Knowledge Infrastructure Databases. Clinical characteristics, chest imaging, bronchoscopic and angiographic findings were summarized. Treatment courses were identified as well as patients' outcome. RESULTS: We report the case of a 41-year-old man, so far in good health, presenting with massive hemoptysis. Bronchoscopy showed blood clots and a protruding lesion covered by mucosa with a white pointed cap at the entrance of the right upper lobe. Biopsies were not attempted. Embolization of bronchial artery was first realized and was not successful, with post procedure complications. Surgical intervention stopped the bleeding and pathological examination of the resected specimen confirmed Dieulafoy's disease of the bronchus. Ninety cases of BDD were reported from 1995 to 2022. The main symptom was hemoptysis. Chest imaging findings were not specific. The diagnosis of BDD was mainly based on the bronchoscopy, branchial angiography and pathological findings or surgical specimens. Bronchoscopy findings were mostly nodular or prominent lesions (52.4%). Twenty-eight patients underwent bronchoscopic biopsies, 20 had massive bleeding and 10 died. Bronchial angiography mainly showed tortuous and dilation of bronchial artery, and the lesions were mainly located in the right bronchus. Selective bronchial artery embolization (SBAE) was performed in 32 patients and 39 patients underwent surgery. CONCLUSION: To our knowledge, this is the first case of bronchial Dieulafoy's disease to be reported in Tunisia and North Africa. When the diagnosis is suspected, bronchoscopic biopsy should be avoided as it might lead to fatal hemorrhage. Selective bronchial artery embolization can stop the bleeding, but surgery can be required.

Assessment Tools for Pulmonary Involvement in Patients with Ankylosing Spondylitis: Is Diaphragmatic Ultrasonography Correlated to Spirometry?

Objective: Spondyloarthritis (SpA) is a chronic inflammatory rheumatic disease that can lead to spinal ankylosis and consequently, restrictive pulmonary dysfunction. Thus, the present study aimed to assess the accuracy of diaphragm ultrasound compared to spirometry in the screening of restrictive pulmonary disorders in radiographic SpA patients. Methods: We conducted a cross-sectional study of 50 patients with radiographic SpA, over six months. Sociodemographic data, clinical characteristics of the disease, as well as biological, radiological, and therapeutic data, were collected. Spirometry and diaphragm ultrasound were performed. Results: The mean age of the study participants (N= 50) was 42.7±11 years [range: 25-66] with male predominance (N= 41). Spirometry showed a restrictive disorder in 32% of cases. The mean chest expansion (CE) value was 3.9±1.81cm [range: 1-9] with a median of 4 cm. A pathological value (<5cm) was observed in 72% of cases. A significant positive correlation was found between the right inspiratory diaphragmatic thickness and forced vital capacity (FVC) (r= 0.36; p = 0.02) and the supine FVC (r=0.29; p = 0.04). The left inspiratory diaphragmatic thickness was correlated with the percentage of the FVC decrease (r= 0.35; p = 0.01) defined as the percentage of difference between FVC and supine FVC. The right expiratory diaphragmatic thickness was associated with the FVC (r=0.32; p = 0.02). A significant positive correlation was found between the CE and the presence of B lines (r=0.32; p = 0.02), but not between the CE and the FVC. Conclusion: The present study showed that diaphragm ultrasonography is correlated with spirometric findings in radiographic SpA patients. Further studies are required to assess its reliability, specificity, and sensitivity in this pathology.

Case Report: Idiopathic pleuroparenchymal fibroelastosis.

Background: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a very rare and a slowly conspicuous progressing chronic lung disease, which usually involves the upper lobes of the lung. This unusual disease, first recognized as a rare idiopathic interstitial pneumonia in 2013, is characterized by dense fibrosis of the visceral pleura and the subjacent lung parenchyma accompanied by elastosis predominating in the subpleural alveolar walls. In the interest of improving our understanding of this uncommon disease, we report a case of IPPFE established by pathology results. Case report: A 73-year-old male patient, smoker, with a medical history of chronic obstructive pulmonary disease, presented since January 2022 with a gradual worsening of dyspnea on exertion and productive cough with weight loss. The chest X-ray detected a thoracic distention. The chest high resolution computed tomography revealed biapical subpleural parenchymatous condensations with tractive bronchiectasis and pleural retraction in the right upper lobe and diffuse bilateral cento-lobular emphysema. A scan-guided trans-parietal lung biopsy showed lung parenchyma tattooed with anthracosic deposits, largely remodeled by fibrous tissue, intermingled with numerous wavy and refractive dyselastotic structures in polarized light. The orcein staining confirmed the presence of excess elastosic fibers within these lesions. All etiological investigations were negative. His lung function studies revealed a reversible obstructive ventilatory disorder. Following a multidisciplinary discussion, the diagnosis of IPPFE was confirmed on the basis of the distribution in the upper lungs on chest computed tomography combined with pathology pattern. Conclusions: This case emphasizes the atypical misleading radiological presentation of IPPFE and the key role of pathological results in establishing the diagnosis. Hence, further studies are needed to improve our understanding of this uncommon disease and to establish clear-cut guidelines for IPPFE diagnosis and management.

Case Report: A very rare case of a Pleural Effusion revealing Multiple Myeloma.

Multiple myeloma is a common malignant bone-based disease. Pleural effusions reported in these patients remain rare. It is commonly due to congestive heart disease, pulmonary embolism, nephrotic syndrome or a second neoplasia. The true myelomatous pleural effusion resulting from a direct tumoral invasion of the pleural are extremely rare. We report here the case of a massive pleural effusion revealing multiple myeloma in a 71-year-old patient. The chest ultrasound showed a massive pleural effusion in the left side with a multinodular thickening of the pleura. The medical thoracoscopy showed a grape-cluster appearance. The diagnosis was made by pleural guided biopsy revealing abnormal plasma cells with an intense positive CD 138 (plasma cell marker) and MUM1 (multiple myeloma oncogene1) staining with a light kappa chain in the protein electrophoresis associated with a myeloma. Unfortunately, our patient died one month after the initial diagnosis. We present also a review of the recent literature in order to highlight the clinical presentations of the myelomatous pleural effusion, the diagnostic tools, the therapeutic strategies as well as the outcomes.

Precision of pleural puncture sites using thoracic ultrasound.

INTRODUCTION: Lung ultrasound (LUS) has been recommended by the British Thoracic Society as a standard of care before performing pleural procedures since 2010. Indeed, the choice of the puncture site based only on physical examination and chest x-ray can lead to complications. The aim of this study was to compare the accuracy of pleural puncture sites using LUS as opposed to clinical examination. METHODS: An evaluative prospective study including 43 patients hospitalized in the pneumology department at the Military Hospital of Tunis was conducted between January and November 2021.Pleural puncture sites were proposed by two groups involving 'senior' and 'junior' physicians, classified according to their experience and grades, based on the clinical examination and the chest x-ray findings. The accuracy of the proposed sites was then verified by an ultrasound-qualified "expert" using LUS. RESULTS: The mean age was 60 ± 17 years. LUS revealed the presence of pleural effusion in 88% of the cases (n=38). Differential diagnosis was therefore excluded in 12% of the cases (n=5), including pleural thickening (5%, n=2) and atelectasis (7%, n=3). Compared to LUS, clinical examination and chest x-ray had lower sensitivities, estimated at 74% and 83%, respectively. The clinical identification error rate was significantly higher in junior (77%) compared to senior physicians (49%) (p<0.05). LUS prevented possible accidental organ puncture in 36% of the cases (n=31). The risk factors associated with inaccurate clinical site selection included right-sided effusion and minimal pleural effusion on chest radiography, with an estimated relative risk (RR) of 1.44 [CI95%:0.56-3.72] and 1.82 [CI95%:0.52-6.40], respectively. The experience of the senior physicians influenced the choice of the clinical sites with moderate agreement (Kappa index: 0.4-0.6). CONCLUSION: Compared to the ACPA- group, the ACPA+ one had more lung-hyperinflation and OVI, and comparative percentages of RVI, MVI, and NSVI. CONCLUSION: LUS significantly improves the accuracy of pleural puncture sites, thus minimizing the risk of complications regardless of the operator's level of clinical experience.

Mental health status of healthcare workers at a third line Tunisian hospital during COVID-19 pandemic.

INTRODUCTION: During the COVID-19(coronavirus-19 disease) pandemic, health care workers (HCWs) faced the risk of infection and distressing work to meet health requirements. The aim of the present stud ywas to evaluate perceptions of HCWs of their security at work in COVID-19 units and their coping strategies, at the military hospital of Tunis, during the second wave of COVID-19. METHODS: A cross-sectional study was conducted via an auto-questionnaire on February 2021.HCWs of the military hospital of Tunis were included. Participants were asked about their perceived security at work in COVID-19 units and their coping behaviors. Mental disorders were assessed, via qualitative questionnaires. RESULTS: A total of 110 HCWs' responses were collected. Fifty-eight participants (52.7%) were females. The mean age was 33.7 years (SD 9.3). They were doctors at 45.5% and nurses at 39.1%. HCWs declared having worked in COVID-19 units in 81.8% of cases. The participants reported their fears about being infected in 58.2% and infecting family members in 85.5% of cases. Protective factors included information about the disease (80%), availability of personal protective equipment (PPE) (80.9%), support from colleagues(72.7%)or superiors at work (50.9%), and ability to communicate with others (63.6%). Depression, anxiety, and insomnia were found in respectively 25.5%, 30%, and 41.8% of HCWs. In multivariate analysis, suffering from a traumatic distress was significantly associated with anxiety and depression (p (p<0.01 and, p<0.05 respectively). In addition, insomnia severity was linked to age (p=0.05) and having colleagues infected with COVID-19 (p<0.05). CONCLUSION: In the present study, HCWs of the military hospital of Tunis stated having high levels of insomnia, anxiety, and depression since the early outbreak of the COVID-19 pandemic. These mental disorders may have a negative impact on the quality of life of HCWs and should be enhanced by psychological support and preventive measures.

Spontaneous Resolution of a Pulmonary Cystic Amyloidosis Mass.

Introduction: Amyloidosis is a rare illness characterized by the deposition in organs of amyloid, which can be detected by histological staining. Amyloidosis restricted to the lower respiratory tract is unusual. Results: We reported the case of a 68-year-old woman with no history of chronic diseasewho presented with dyspnoea on exertion, cough and fatigue. The physical examination was unremarkable. A CT scan showed a cystic mass with a thickened wall in the apical segment of the left lower lobe. A biopsy of the mass was performed, and histological and immunohistochemical study confirmed the diagnosis of AL amyloidosis. The patient's clinical and radiological symptoms spontaneously improved without treatment after 3 years. Conclusion: Amyloid-related cystic lung disease is a rare presentation of amyloidosis in the thorax. More case reports are required to determine if such masses can resolve without treatment and whether amyloid-associated cystic lung disease actually exists. LEARNING POINTS: Dyspnoea and cough are a rare atypical presentation that can reveal pulmonary amyloidosis.A cystic lung mass should raise suspicion for pulmonary amyloidosis.Clinical symptoms and radiological findings of a cystic mass spontaneously resolved without treatment after 3 years in this patient with pulmonary amyloidosis.

Parsonage-Turner syndrome of the brachial plexus secondary to COVID-19 vaccine: A case report.

Parsonage-Turner syndrome (PTS) is a peripheral inflammatory neuropathy of unknown etiology. We present a rare case of a 50-year-old male patient with PTS post-COVID-19 BNT162b2 mRNA vaccine. Symptoms occurred 15 days after the second dose. He was treated with corticosteroids, analgesics, and physical rehabilitation with a partial recovery.

[Comparative study of patients hospitalized for SARS-CoV-2 infection in two consecutive waves in Tunisia]. / Étude comparative de patients hospitalisés pour une infection SARS-CoV-2 au cours de deux vagues consécutives en Tunisie.

Introduction: Since December 2019, a novel coronavirus (SARS-CoV-2) has triggered a global pandemic with a heavy medical and societal-economic toll. The health consequences were not similar during the successive waves that affected several countries. The aim of our study was to compare the sociodemographic, clinical and evolutionary features of COVID-19 patients hospitalized at the Military Hospital of Tunis (HMPIT) during the 2nd and 3rd waves that affected the country. Patients and methods: Observational prospective study involving 1,527 COVID-19 patients hospitalized at HMPIT over 11 months, divided into two periods: from July 2020 to December 2020 called the second wave (V2) and from January 2021 to May 2021 called the third wave (V3). We compared the epidemiological data, the clinical form and the evolution of the patients for each period. Results: The number of hospitalized patients was 636 during V2 compared to 891 during V3. Average age was 63.5 ± 15.3 years during V2 versus 65.8 ± 17.8 years during V3 (P = not significant [NS]). The percentage of young adults [18-40 years] was 6.5% during V2 compared to 6.7% during V3 (P = NS). The gender ratio (M/F) was 1.59 for V2 and 1.42 for V3 (P = NS). Comorbidities were present in 65% of V2 patients and 66.3% of V3 patients (P = NS), with hypertension being the most prevalent one in both groups (47.2% for V2 versus 44.9% for V3; P = NS), followed by overweight, dyslipidemia and diabetes (33% for V2 versus 39.3% for V3; P = 0.012). The median duration between symptoms onset and hospitalization was 7 days [5-10] during V2 versus 8.5 days during V3 [5-12] (P = 0.0004). The severe clinical form was present in 49% of patients admitted during V2 compared to 34.8% during V3 (P < 10-3). The critical form represented 18.6% of cases during V2 against 16.8% during V3 (P = NS). The average hospital length of stay in COVID units (outside of intensive care unit) was 8.4 ± 5.4 days during V2 and 9.8 ± 5.7 days during V3. The average length of stay was significantly longer for the intensive care unit (11.3 ± 3.4 days for V2 versus 13.8 ± 3.9 days for V3; P = 0.01). The case fatality rate was 24.5% during V2 and 20.7% during V3 (P = NS). Median age of death was 70.2 years [42-88] during V2 and 70.4 years [22-96] during V3 with 2 patients less than 40 years of age (1%) for the latter period. The gender ratio (M/F) of deceased patients was 3.21 for V2 and 1.5 for V3 (P = 0.001). The case fatality rate was higher in the intensive care unit (65.4% for V2 versus 69.7% for V3; P = NS). Causes of death were dominated by ARDS (acute respiratory distress syndrome) for both periods (55.1% for V2 versus 70.8% for V3; P = 0.002), followed by septic shock (12.8% for V2 versus 10.8% for V3; P = NS) and multi-organ failure (9.6% for V2 versus 7.0% for V3; P = NS). Conclusion: This study revealed a decrease in severe and critical clinical forms during the 3rd wave, as well as a decrease in the case fatality rate compared to the previous wave, due to improved management and vaccination. On the other hand, the percentage of ARDS was significantly higher during this wave probably related to the beginning of circulation in our country of the Delta variant causing more severe clinical cases.

Massive Haemoptysis Treated with Bronchial Artery Embolisation in COVID-19 Infection.

Background: Massive haemoptysis is a rare symptom ofcoronavirus disease 2019 (COVID-19). Management can be very challenging due to the lack of clear recommendations. Case description: We report a case of massive recurrent haemoptysis in a young patient who tested positive for COVID-19 with successful management using endovascular embolization. Discussion: Life-threatening massive haemoptysis has rarely been reported as the only manifestation of COVID-19. Embolisation was the therapeutic option chosen to manage this emergency. LEARNING POINTS: Haemoptysis is a rare atypical presentation that can reveal COVID-19, highlighting the complexity of its pathogenesis.Atypical manifestations should raise suspicion for COVID-19.In this patient with COVID-19, life-threatening massive haemoptysis was successfully treated with endovascular embolisation.

A recurrent side-changing febrile pleural effusion revealing familial Mediterranean fever: a case report.

Familial Mediterranean Fever (FMF), characterized by recurrent polyserositis, is an autosomal recessive disease involving essentially Mediterranean populations. We report the case of a 30-year-old Tunisian military patient complaining of fever and chest pain recurring on board a Navy military vessel, due to side-changing pleural effusion. On landing, a marked improvement of symptoms was noticed. Gene testing was performed when the diagnostic survey ruled out common etiologies, revealing a homozygous mutation of the FMF gene type M680l/M680l. The prescription of colchicine and the exemption from boarding led to the resolution of the symptoms with no recurrence of pleural effusion. Therefore, the diagnosis of FMF should be considered in a context of a recurrent pleural effusion in the youth, with a negative etiological assessment, notably in an ethnic group at risk. Thus, early diagnosis and adequate treatment may prevent the development of secondary amyloidosis, a serious complication of FMF.

Case Report: The first reported case of pulmonary alveolar proteinosis with myasthenia gravis in a 27-year-old patient.

Background: Pulmonary alveolar proteinosis is a very rare diffuse lung disease characterized by the accumulation of amorphous and periodic acid Schiff-positive lipoproteinaceous material in the alveolar spaces due to impaired surfactant clearance by alveolar macrophages. Three main types were identified: Autoimmune, secondary and congenital. Pulmonary alveolar proteinosis has been previously reported to be associated with several systemic auto-immune diseases. Accordingly, we present the first case report of pulmonary alveolar proteinosis associated with myasthenia gravis. Case: A 27-year-old female patient, ex-smoker, developed a dyspnea on exertion in 2020. The chest X-ray detected diffuse symmetric alveolar opacities. Pulmonary infection was ruled out, particularly COVID-19 infection. The chest scan revealed the "crazy paving" pattern. The bronchoalveolar lavage showed a rosy liquid with granular acellular eosinophilic material Periodic acid-Schiff positive. According to the lung biopsy results, she was diagnosed with pulmonary alveolar proteinosis. The granulocyte macrophage colony-stimulating factor autoantibodies were negative. Nine months later, she was diagnosed with bulbar seronegative myasthenia gravis, confirmed with the electroneuromyography with repetitive nerve stimulation showing significant amplitude decrement of the trapezius and spinal muscles. She was treated with pyridostigmine, oral corticosteroids and azathioprine. Given the worsening respiratory condition of the patient, a bilateral whole lung lavage was performed with a partial resolution of symptoms. Thus, this previously unreported association was treated successfully with rituximab, including improvement of dyspnea, diplopia and muscle fatigability at six months of follow-up. Conclusions: This case emphasizes on the possible association of auto-immune disease to PAP, which could worsen the disease course, as the specific treatment does not exist yet. Hence, further studies are needed to establish clear-cut guidelines for PAP management, particularly when associated to auto-immune diseases.

Second lung malignancy and Richter syndrome in chronic lymphocytic leukemia: case report and literature review.

BACKGROUND: Chronic lymphocytic leukemia (CLL) is the most frequent lymphoproliferative disease. Transformation into Richter disease and occurrence of second malignancies involving the lungs are rare complications. The hallmarks of any thoracic involvement are still unknown. CASE PRESENTATION: We report a case of a 56-year-old male patient, with history of tobacco smoking, who presented with recurrent hemoptysis, fatigue and weight loss. Physical examination was normal except a slightly enlarged supraclavicular lymph node. Chest x-ray revealed a mediastinal widening due to enlarged paratracheal nodes and a left parahilar infiltrate. Blood tests showed a hyperlymphocytosis and a biological inflammatory syndrome. CT scan showed bilateral mediastinal and axillary lymphadenopathy, as well as left supraclavicular lymphadenopathy, with a left upper lobe alveolar attenuation and a solitary contralateral pulmonary nodule. Examination of Virchow's node and bone marrow biopsies confirmed metastasis of a pulmonary adenocarcinoma, as well as chronic lymphocytic leukemia with Richter's transformation. The clinical course was unfavorable since the first days of therapy as the patient passed away in a matter of a few days. CONCLUSIONS: Steady surveillance of CLL patients and systematic screening for second solid tumors, particularly lung cancer, and Richter's transformation seem to be relevant more than ever. Early diagnosis might help us understand the pathways leading to these complications and adapt therapy.

Spontaneous resolution of a tumor like pulmonary sarcoidosis.

We report a case of thoracic sarcoidosis in a 72-year-old female, snuff taker, who presented with multinodular pulmonary lesions on chest x-ray. Clinical and biological findings were poor. Thoracic imaging showed soft tissue density nodules with irregular borders. The diagnosis of 'cannon ball' metastases was suspected. A thorough investigation strategy could not prove malignancy. A complete radiologic clearing was obtained spontaneously within three months. A rereading of pathology slides performed afterwards showed multinucleated giant cells on hemorrhagic background with a lymphocytic alveolitis. The diagnosis of pseudotumoral sarcoidosis was made. The clinical course was favorable with a 6years follow-up.