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.

Pulmonary aspergilloma with prominent oxalate deposition.

Some Aspergillus species produce oxalic acid, which reacts with tissue calcium or blood to precipitate calcium oxalate. Oxalate crystals can induce lung and kidney damage. The presence of oxalate crystals can suggest the diagnosis of aspergillosis, even when Aspergillus hyphae are absent on microscopic slides.

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.

Case Report: Allergic Bronchopulmonary Aspergillosis Revealing Asthma.

Allergic bronchopulmonary aspergillosis (ABPA) is an immunological pulmonary disorder caused by hypersensitivity to Aspergillus which colonizes the airways of patients with asthma and cystic fibrosis. Its diagnosis could be difficult in some cases due to atypical presentations especially when there is no medical history of asthma. Treatment of ABPA is frequently associated to side effects but cumulated drug toxicity due to different molecules is rarely reported. An accurate choice among the different available molecules and effective on ABPA is crucial. We report a case of ABPA in a woman without a known history of asthma. She presented an acute bronchitis with wheezing dyspnea leading to an acute respiratory failure. She was hospitalized in the intensive care unit. The bronchoscopy revealed a complete obstruction of the left primary bronchus by a sticky greenish material. The culture of this material isolated Aspergillus fumigatus and that of bronchial aspiration fluid isolated Pseudomonas aeruginosa. The diagnosis of ABPA was based on elevated eosinophil count, the presence of specific IgE and IgG against Aspergillus fumigatus and left segmental collapse on chest computed tomography. The patient received an inhaled treatment for her asthma and a high dose of oral corticosteroids for ABPA. Her symptoms improved but during the decrease of corticosteroids, the patient presented a relapse. She received itraconazole in addition to corticosteroids. Four months later, she presented a drug-induced hepatitis due to itraconazole which was immediately stopped. During the monitoring of her asthma which was partially controlled, the patient presented an aseptic osteonecrosis of both femoral heads that required surgery. Nine months after itraconazole discontinuation, she presented a second relapse of her ABPA. She received voriconazole for nine months associated with a low dose of systemic corticosteroid therapy with an improvement of her symptoms. After discontinuation of antifungal treatment, there was no relapse for one year follow-up.

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.