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.
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.
The presence of associated oxalosis in a given Aspergillus infection should be stated in the pathology report because of its potential clinical significance and diagnostic value.
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.
Une tumeur endobronchique déroutante Snène Houda; Khalil, Zayen; Salah Nozha Ben ...
The Pan African medical journal,
10/2020, Letnik:
37, Številka:
201
Journal Article
Recenzirano
Odprti dostop
Le cancer broncho-pulmonaire représente la première cause de décès par cancer chez l´homme et le deuxième chez la femme. Certaines présentations endoscopiques ou radiologiques peuvent orienter le ...diagnostic histologique et ainsi faciliter la prise en charge thérapeutique. Nous rapportons l´observation d´un homme de 54 ans, tabagique, coronarien récemment stenté, consultant pour hémoptysie et aggravation de sa dyspnée évoluant depuis un mois. Sa radiographie du thorax avait objectivé une hyperclarté de l´hémichamp pulmonaire gauche avec des signes de rétraction. A la fibroscopie bronchique, il existait une formation bourgeonnante framboisée, saignant spontanément, accouchée par la bronche souche gauche évoquant une tumeur carcinoïde. Le scanner thoracique avait objectivé un processus tissulaire endoluminal, au niveau de la bronche souche gauche situé à quatre cm de la carène, peu rehaussé au produit de contraste et compliqué d´atélectasie. Une chirurgie diagnostique et thérapeutique a permis de redresser le diagnostic en faveur d´un hamartochondrome endobronchique.
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.
Background
COVID-19 disease has spread rapidly worldwide, causing high mortality. Accessible biomarkers capable of early identification of patients at risk of severe form are needed in clinical ...practice. The aim of the study was to determine the biological markers that predict a critical condition.
Methods
Retrospective study including patients with confirmed COVID-19 hospitalized between September 2020 and June 2021. The primary endpoint was progression to critical status within 7 days from admission. We defined two groups:
Critical group: Patients who developed a critical condition or died or transferred to the ICU before or at 7
th day.
Non-critical group: Patients who remained in non-critical respiratory status until 7
th day or discharged before or at 7
th day.
Results
Our study included 456 patients, with a sex ratio of 1.32 and an average age of 62 years. At the 7
th day of hospitalization, 115 (25.2%) patients were in the critical group and 341 (74.8%) patients were in the non-critical group. The univariate logistic regression indicated that laboratory findings between non-critical and critical groups showed that C-reactive protein (CRP) (p=0.047), D-Dimer (p=0.011), creatinine (0.026), creatine kinase (p=0.039), lactate dehydrogenase (p=0.04), and troponin (p=0.001) were all higher among patients in critical group. However, lymphocyte (p<0.001) and platelet (p<0.001) counts were significantly lower among the critical group. Multivariate logistic regression model, identified four independent risk factors: lymphopenia (OR=2.771, 95%CI=1.482-5.181, p=0.001), Neutrophil to Lymphocyte Ratio (OR=2.286, 95%CI=1.461-3.578, p<0.001), thrombocytopenia (OR=1.944, 95%CI=1.092-3.459, p=0.024), and CRP>71.5 (OR=1.598, 95% CI=1.042-2.45, p=0.032) were associated to critical group.
Conclusions
Our results show the predictive value of some biological markers to evaluate the prognosis of COVID-19 pneumonia. A prognostic score could be proposed for guiding clinical care and improving patient outcomes.
Introduction :
La broncho-pneumopathie chronique obstructive (BPCO) est une pathologie pulmonaire chronique dont le retentissement est multi-systémique, pouvant se compliquer de troubles ...psycho-affectifs.
Objectif :
Estimer la fréquence de l’anxiété et de la dépression chez les patients suivis pour BPCO à l’état stable dans un CHU à Tunis et de déterminer les facteurs prédictifs de leur apparition.
Méthodes :
Il s'agissait d'une étude transversale à visée analytique réalisée auprès de patients suivis pour une BPCO à l’état stable aux consultations externes du service de pneumologie du CHU Mongi Slim La Marsa. Les symptômes de la BPCO ont été évalués par le Chronic obstructive pulmonary disease Assessment Test (CAT),sa sévérité par la classification du GOLD 2020 et le dépistage des troubles psycho-affectifs par l’échelle HAD.
Résultats :
Cent trois patients ont été colligés, d’âge moyen de 64 ans. La prévalence de l’anxiété était de 44,7% et celle de la dépression de 33,9% avec 26,2% des patients ayant un épisode dépressif majeur. En analyse multivariée par régression logistique binaire, les valeurs les plus élevées du CAT étaient associées à l’anxiété, à la dépression et l’apparition d’un épisode dépressif majeur. Les autres facteurs associés à l’anxiétéet à l’apparition d’un épisode dépressif majeur étaient les troubles du rythme cardiaques associés et les valeurs les plus basses de l’IMC. De plus, le sexe féminin était associé à l’apparition d’un épisode dépressif majeur.
Conclusion :
L’anxiété et la dépression sont fréquentes chez les patients Tunisiens souffrant de BPCO, d’où tout l’intérêt de leur dépistage systématique.
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.
L´encéphalite limbique (EL) est une pathologie rare souvent d´origine paranéoplasique avec une association fréquente au cancer broncho-pulmonaire. Son diagnostic est radiologique, reposant sur ...l´imagerie par résonnance magnétique (IRM) cérébrale. Nous rapportons le cas d´une femme âgée de 54 ans, tabagique, qui consulte pour toux sèche trainante. La radiographie du thorax avait montré une opacité pulmonaire droite suspecte. La fibroscopie bronchique et les biopsies bronchiques n´étaient pas contributives. Au scanner thoraco-abdomino-pelvien et cérébral, il existait une masse du lobe supérieur droit classée T4N2M1a. Une biopsie pulmonaire scanno-guidée a confirmé le diagnostic d´un adénocarcinome broncho-pulmonaire. Au cours de son exploration, la patiente avait rapporté la notion de troubles récents de la mémoire avec une humeur dépressive, une anxiété et une confusion paroxystique. Les bilans métaboliques et infectieux étaient normaux et l´IRM cérébrale était en faveur d´une EL. L´évolution a été rapidement progressive et la patiente est décédée en une dizaine de jours.
PDF
