A rare coexistence of sarcoidosis with overlap syndrome and sarcoidosis with primary biliary cholangitis and Sjogren's syndrome: Two distinct case reports.

Sarcoidosis and the overlap syndrome of autoimmune hepatitis and primary biliary cholangitis (PBC) share common clinical, biological, and histological features. The simultaneous occurrence of these diseases have been reported in few cases and suggests that a common pathway which may contribute to granuloma formation in both conditions. We report the cases of two female patients having an association of sarcoidosis and inflammatory liver diseases. The first case is of a 61-year-old woman had been monitored for an overlap syndrome of PBC and autoimmune hepatitis (AIH). Therefore, treatment with azathiprine has been initiated associated with ursodeoxycholic acid (UDCA). Azathioprine had to be discontinued due to digestive intolerance, specifically chronic diarrhea and abdominal pain. The patient remained clinically stable on UDCA and her liver function tests were stable for years, until she developed symptoms of progressive dyspnea without any other associated signs. Chest computed tomography (CT) revealed mediastinal enlargement, bilateral pulmonary nodules, and symmetrical adenomegalies in the mediastinum. The bronchoalveolar lavage (BAL) revealed increased cellularity, with a notable elevation in lymphocyte count (48 %) and a CD4/CD8 ratio of 4. The patient underwent mediastinoscopy; a biopsy of the right laterotracheal (4R) adenomegaly was performed. Histological examination of the lymph node showed epithelioid and giant-cell tuberculoid lymphadenitis without necrosis, compatible with sarcoidosis. Ophthalmological and cardiac assessments were normal. Plethysmography test was normal and there was no need for corticosteroid treatment; a surveillance was planned. Treatment with UDCA was pursued. The second case is of a 50-year-old woman with no medical history presented symptoms including dry eyes and mouth, inflammatory-type polyarthralgia affecting knees and wrists, bilateral Raynaud's phenomenon, right hypochondrium pain, and worsening dyspnea over six months. Liver analysis showed elevation of alkaline phosphatase (ALP) to three times upper limit of normal (ULN) and gamma-glutamyltransferase (GGT) to 5 times ULN. This cholestasis was associated with an increase in transaminase activity to 5 times ULN for over six months. Immunological tests revealed positive anti-nuclear antibodies (ANA), anti-Ro52, anti-M23E, and anti-centromere antibodies. Chest-CT showed multiple bilateral bronchiolar parenchymal micronodules mostly in the upper and posterior regions without any mediastinal adenomegaly. Bronchial endoscopy was normal, and biopsies indicated chronic inflammation. The BAL revealed increased cellularity, characterized by a high lymphocyte count (51.7 %) and a CD4/CD8 ratio of 2.8. Biopsy of minor salivary gland revealed grade 4 lymphocytic sialadenitis. Skin biopsy revealed an epithelioid granuloma without caseous necrosis. Liver biopsy performed in the presence of cytolysis and moderate hepatic insufficiency, revealed granulomatous hepatitis and cholangitis lesions along with septal fibrosis suggestive of PBC. The diagnosis of cutaneous and pulmonary sarcoidosis with PBC and Sjögren's syndrome was retained. The spirometry and diffusing capacity for carbon monoxide value were normal. Treatment involved UDCA, corticosteroids, and azathioprine, leading to clinical and biological improvement. Sarcoidosis shares some clinical manifestations with autoimmune liver diseases, primarily PBC. A hepatic granuloma with a different appearance and location can guide the diagnosis. Early diagnosis and appropriate management can avoid serious complications and improve prognosis.

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.

[Paraneoplastic limbic encephalitis complicating pulmonary adenocarcinoma: a case report]. / Encéphalite limbique paranéoplasique compliquant un adénocarcinome pulmonaire: à propos d'un cas.

Limbic encephalitis (LE) is a rare disease often of paraneoplastic origin. It is frequently associated with bronchopulmonary cancer. Diagnosis is based on brain magnetic resonance imaging (MRI). We here report the case of a 54-year-old female patient with a history of active smoking, presenting with chronic dry cough. Chest X-ray showed suspicious right lung opacity. Bronchial fibroscopy and bronchial biopsies were not contributory. Thoraco-abdomino-pelvic computed tomography (CT)-scan and brain CT-scan showed a mass in the right upper lobe classified as T4N2M1a. CT-guided lung biopsy confirmed the diagnosis of bronchopulmonary adenocarcinoma. The patient had reported a recent history of memory disturbances associated with depressed mood, anxiety and paroxysmal confusion. Metabolic screening and tests for detecting infection were normal and brain MRI suggested limbic encephalitis. The evolution was characterized by rapid disease progression; the patient died in about ten days.

COPD patients' body composition and its impact on lung function.

INTRODUCTION: Despite fat-free mass index (FFMI) is one of the strongest predictive factors of survival during chronic obstructive pulmonary disease (COPD), there is a considerable lack of information regarding body composition in Tunisian patients with COPD.   Aim: Describe the body composition of Tunisian patients followed for COPD and examine the relationship between body composition and the severity of the disease. METHODS: Cross-sectional study of patients with stable COPD. Body composition was assessed by bioelectrical impedance analysis. Pulmonary function tests (PFT) included spirometry with plethysmography and the six-minute walking test. The severity of dyspnea was assessed by the mMRC scale. RESULTS: During the study period, 104 patients with stable COPD were included (average age= 65.9 years and average FEV1= 49.3%). Fifty-four percent of patients were GOLD D stage. According to the IMM, malnutrition was identified in 20.2% of cases. Patients with low FFMI were the most symptomatic, had a more severe air flow limitation and a more severe disease. The walking distance was lower in malnourished patients. However, FFMI was not significantly associated with exercise capacity. CONCLUSIONS: Malnutrition is highly prevalent in COPD patients and is correlated to the severity of the disease. Thus, body composition analysis should be considered in COPD patient management.

Anxiety and depression in Tunisian patients with COPD.

BACKGROUND: Chronic obstructive pulmonary disease (COPD) is a chronic lung disease with a multi-systemic impact that can be complicated by psycho-affective disorders. AIM: To determinethe frequency of anxiety and depression in patients followed for stable COPD in a university hospital-center in Tunisand to determine the predictors of their onset. METHODS: This was an analytical cross-sectional studyof patients followed for stable COPD at the pulmonology department of the Mongi Slim La Marsa Hospital. The symptoms of COPD were assessed by the Chronic obstructive pulmonary disease Assessment Test (CAT), its severity by the GOLD 2020 classification and the screening of psycho-affective disorders by the HAD scale. RESULTS: One hundred and three patients were enrolled, with an average age of 64 years. The prevalence of anxiety was 44.7% and that of depression was 33.9%with 26.2% of patients with a major depressive episode. In multivariate analysis by binary logistic regression, the highest values of CAT score were associated with anxiety, depression, and the onset of a major depressive episode. Other factors associated with anxiety and the onset of a major depressive episode were associated heart rhythm disorders and the lowest BMI values. In addition, the female gender was associated with the onset of a major depressive episode. CONCLUSION: Anxiety and depression are common in Tunisian patients with COPD, hence the interest of their systematic screening.

[Confusion in the diagnosis of endobronchial tumor]. / Une tumeur endobronchique déroutante.

Bronchopulmonary cancer is the leading cause of death in men and the second in women. Some endoscopic or radiological features may guide histological diagnosis and thus facilitate therapeutic management. We here report the case of a 54-year old man, with a history of smoking and recent coronary stent implantation, presenting with haemoptysis and worsening of dyspnea which had evolved over the last month. Chest x-ray showed left pulmonary hemifield lucency with signs of retraction. Bronchial fibroscopy objectified raspberry bud formation spontaneously bleeding, originating from the left main bronchus and suggesting carcinoid tumor. Chest computed tomography (CT) scan showed poorly enhanced endoluminal tissue process at the level of the left main bronchus, located four cm from the carina and complicated with atelectasis. Diagnostic and therapeutic surgery helped to adjust to a diagnosis of endobronchial amartocondroma.

Esophageal leiomyomas presenting as a mediastinal mass.

Although benign tumors of the esophagus are very rare, the leiomyomas are frequently recorded. Most of them are small, asymptomatic and without risk of malignant degeneration. These benign tumors are usually discovered fortuitously on endoscopy. Sometimes, they may manifest clinically by dysphagia, hematemesis or other signs. They may mimic the esophageal cancer, which is more frequent, or some mediastinal tumors. The diagnosis can be oriented by the barium swallow esophagogram or other imaging methods, yet, only the histological examination gives the confirmation of the diagnosis. We report the case of a 50-year-old man, non-smoker, complaining of dysphagia, epigastric pain and deterioration of general condition. The clinical and radiological presentation mimicked a mediastinal tumor. Surgery was performed, and histological examination concluded to two leiomyomas of the esophagus.

The Z-score: A new tool in the interpretation of spirometric data.

Spirometry is an important tool in the diagnosis and management of patients with respiratory pathology. An appropriate interpretation of the spirometric data requires the use of a population-specific reference equation. However, the most widely used equations were established in European populations with limited age groups. The extrapolation of these equations, based on a specific population, and their uses for a different population led to measurement and interpretation biases. In 2012, an international working group conducted a multicenter study and published new reference equations called The Global Lung Initiative (GLI). These enabled the modeling of spirometric parameters from a very large sample collected in several ethnic groups using modern statistical techniques to establish continuous equations for all ages and in many countries. The GLI also recommends the use of a new statistical tool for the expression of results: The Z-score. This tool allows to express, in a simple way: how many standard deviations a subject is deviated from its reference value. The Z-score is calculated by the ratio of the difference between the measured value and that predicted with the residual standard deviation. This simple approach has reduced the false positive results found by the use of the conventional limits of 80% compared to a predicted value or 0.70 in absolute value for the definition of bronchial obstruction that remain still used.

Preoperative assessment in pulmonary resection surgery.

Pulmonary resection can be associated with a significant risk of morbidity and mortality, which depends on the nature and extent of pulmonary resection but also on the patient himself. This risk can be apprehended by a preoperative assessment which estimates the immediate operative risk as well as the physiological state and the post-operative quality of life which can require more conservative therapies. Currently, preoperative exploration of a patient is based on various technological tools, which can range from simple electrocardiogram or simple spirometry to a complex exploration such as a cardiorespiratory effort test. These multiple evaluation tools require the rationalization of good practice processes according to international recommendations, taking into account the patient's specificity and the country context. This approach makes it possible to prioritize examinations according to their availability and accessibility in order to identify patients with high operative risk and to offer them an appropriate therapeutic choice.

Childhood tuberculosis: A descriptive study in a pneumo-pediatrics department in Tunisia.

Objective to assess the different localizations of tuberculosis (TB) in children in a pneumopediatric department in Tunisia and to describe its diagnosis tools since clinical investigations of childhood TB are challenged by the paucibacillary nature of the disease and the difficulties in obtaining specimens. Methods Forty-six cases of TB in children were studied between 2008 and 2013. Clinical history, examination and chest radiography were reported. Several investigations have been conducted to confirm the diagnosis of TB such as: tuberculin skin test (TST), bacteriological and histological investigations. Anti-tuberculosis treatment was prescribed according to the national guidelines. Results Cough and deterioration in general condition were the most frequent symptoms (47.8% and 43.7%). The other children presented cervical swelling (19.5%), chest pain (17.4%) and hemoptysis (4.3%). Abnormalities have been found in chest radiography in 35 cases (76%). TST was positive in 73% of cases. Diagnosis of TB was confirmed in 56.6% of cases by Mycobacterium tuberculosis (MT) isolation and/or biopsy. The diagnosis was made on presumptive arguments in 20 cases (43.4%) based on a history of TB contact, suggestive symptoms and a positive TST. A surgical biopsy was necessary for diagnosis in 17 cases (nasopharynx, bone, cervical, mediastinal and mesenteric lymph nodes). Pulmonary TB was diagnosed in 52% of cases. Two children were diagnosed with disseminated TB. A diagnosis delay was noted with an average of 20 days and a contact history was found in 52% of the children. All children were treated according to the national guidelines without major side effects. Healing without sequelae was achieved in 91% of cases. Conclusion Children represent a population at high risk for TB especially after a household contact with a higher frequency of multifocal forms compared to adults. The difficulty of the diagnosis in children may explain partially the diagnosis delay, but efforts must be done to improve prevention and diagnosis in our country.