scholarly journals Pulmonary Amyloidosis with Multiple Cystic Lesions with Central Calcifications

Author(s):  
Forster C ◽  
◽  
Bénière C ◽  
Perentes JY ◽  
Christodoulou M ◽  
...  

A non-smoker 80-year old woman with a past medical history of inactive and untreated systemic lupus erythematous diagnosed in 1983 and polymyalgia rheumatica treated with prednisone (3 mg once daily) since 2018, was referred to our emergency department because of left-sided chest pain and dyspnoea. She presented no cough, weight loss or fever and there was no history of Sjogren’s syndrome. Complete blood count was unremarkable and no inflammatory syndrome was observed. A chest CT-scan revealed multiple diffuse cystic parenchymal lesions with thin walls and central nodular calcifications in both lungs (Figure 1A). The sputum culture was negative for mycobacterium tuberculosis, legionella pneumophilia, mycoplasma pneumonia, chlamydia pneumonia, coronavirus, echinococcosis and aspergillus. Anti-Nuclear Antibody (ANA) and anti-Ku tests were positive whereas anti-neutrophil cytoplasmic antibody (ANCA) and anti-nucleoprotein tests were negative. The preoperative pulmonary function tests showed a FEV1 of 78% and a DLCO of 73% of the predicted values.

2019 ◽  
Vol 45 (5) ◽  
Author(s):  
Ana Beatriz Hortense ◽  
Marcel Koenigkam dos Santos ◽  
Danilo Wada ◽  
Alexandre Todorovic Fabro ◽  
Mariana Lima ◽  
...  

ABSTRACT Objective: To describe the clinical, functional, and radiological features of index cases of familial pulmonary fibrosis (FPF) in Brazil. Methods: We evaluated 35 patients with FPF - of whom 18 (51.4%) were women - with a median age of 66.0 years (range, 35.5-89.3 years). All of the patients completed a standardized questionnaire, as well as undergoing pulmonary function tests and HRCT of the chest. In 6 cases, lung tissue samples were obtained: from surgical biopsies in 5 cases; and from an autopsy in 1 case. Results: A history of smoking and a history of exposure to birds or mold were reported in 45.7% and 80.0% of the cases, respectively. Cough and marked dyspnea were reported by 62.8% and 48.6% of the patients, respectively. Fine crackles were detected in 91.4% of the patients. In 4 patients, the findings were suspicious for telomere disease. The median FVC and DLCO, as percentages of the predicted values, were 64.9% (range, 48.8-105.7%) and 38.9% (range, 16.0-60.0%), respectively. Nine patients had reduced DLCO despite having normal spirometry results. Regarding HRCT, patterns typical of usual interstitial pneumonia were found in 6 patients (17.1%). In 25 cases (71.5%), the HRCT features were consistent with a diagnosis other than idiopathic pulmonary fibrosis. In 11 cases (31.4%), the radiological patterns were uncharacteristic of interstitial lung disease. Of the six lung tissue samples analyzed, four showed interstitial pneumonia with bronchiolocentric accentuation, and, on the basis of the clinical and radiological data, the corresponding patients were diagnosed with hypersensitivity pneumonitis. Conclusions: Patients with FPF can present with a wide variety of clinical features. Most HRCT scans of these patients exhibit patterns not typical of usual interstitial pneumonia. The family history of fibrotic lung diseases should be investigated in all patients under suspicion, regardless of their age.


2021 ◽  
Vol 3 (1) ◽  
pp. 01-03
Author(s):  
Animasahun Ba

Paediatric multisystem inflammatory syndrome (PMIS) occurs two to four weeks following exposure to the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the virus responsible for coronavirus 2019 (COVID-19). It is characterized by the presence of persistent fever, inflammation and organ dysfunction. We present AE a 10-year old Nigerian boy who presented to the children emergency room with a history of continuous and high grade fever with back and multiple joint pains. While on admission he developed cough, difficulty with breathing, bleeding from the gums and altered consciousness which progressed to loss of consciousness. Complete blood count showed neutrophilia of 82.9%, lymphopenia of 9.8%, thrombocytopenia of 142,000 cells/mm3, elevated erythrocyte sedimentation rate of 75mm/hr, chest radiograph findings of ground glass appearance and consolidation in all lung lobes bilaterally with a positive reverse transcriptase-polymerase chain reaction test for COVID-19 which met the Centre for Disease Control criteria for the diagnosis of pediatric multisystem inflammatory syndrome. The patient was treated with intranasal oxygen, transfused with fresh whole blood, analgesics and antifailure drugs. His condition however deteriorated while on admission and died on the seven days of admission.


2021 ◽  
Vol 2 (2) ◽  
pp. 038-041
Author(s):  
A Ekladious ◽  
L Fish ◽  
C De Chaneet ◽  
C Cox

A 75-year-old man presented with pleuritic chest pain, haemoptysis and dyspnoea. Imaging found multiple pulmonary nodules, concerning for malignancy. CT-guided biopsy was consistent with amyloid. The patient has a history of pulmonary amyloidosis, with a single nodule resected 14 years prior. This case allows comparison between imaging fourteen years apart, providing insight into the progressive nature of these benign nodules. Nodular pulmonary amyloidosis is a rare condition with few case reports published. Of those published, few are of nodular amyloidosis in the absence of underlying neoplastic aetiology. This case presents a 14-year interval of a patient with nodular amyloidosis, allowing insight into disease progression which has not previously been well described. A 75-year-old man presented to a regional hospital in Australia with right sided pleuritic chest pain, and a 2-week history of productive cough, haemoptysis, dyspnoea and reduced exercise tolerance. Further questioning revealed a 3-month history of worsening dyspnoea, haemoptysis and cough, with no orthopnoea, paroxysmal nocturnal dyspnoea or weight loss. The patients’ past medical history included resection of an amyloid tumour from the left lower lobe of the lung in 2004, ex-smoker with a 30-pack year history, significant occupational asbestos exposure through work as a diesel mechanic, and hypercholesterolaemia. The patient lives at home with his partner and is independent with the activities of daily living. On examination, the patient appeared well, in no respiratory distress. Observations were within normal limits, with oxygen saturations of 94% on room air, respiration rate of 18. On auscultation, there were late inspiratory crackles, as well as decreased hepatic dullness to percussion. There was a central trachea and no palpable cervical lymphadenopathy. Cardiovascular examination was unremarkable, and the patient was euvolaemic. Abdominal examination was unremarkable with no palpable organomegaly, and he did not have a rash or skin abnormalities seen. The initial investigations were conducted to investigate a potential lung malignancy underlying malignancy, based on the appearance on CT scan. The patient advised us that he had a previous diagnosis of primary pulmonary amyloidosis, first diagnosed 14 years ago. The diagnosis was made after a chest x-ray and CT showed a nodule, suspicious for cancer. Pulmonary function tests were performed and he was referred to a Cardiothoracic surgeon for consideration of a left lower lobectomy. He was treated with a wedge resection. Histopathology from this specimen showed primary pulmonary amyloidosis. There was a delay in the understanding of how the patient came to have this rare diagnosis, as it took five days for the medical records to be sourced from the archive.


2019 ◽  
Vol 31 (2) ◽  
pp. 261-264
Author(s):  
Karim Mithani ◽  
Ying Meng ◽  
David Pinilla ◽  
Nova Thani ◽  
Kayee Tung ◽  
...  

A 52-year-old man with a 10-year history of treatment-resistant asthma presented with repeated exacerbations over the course of 10 months. His symptoms were not responsive to salbutamol or inhaled corticosteroid agents, and he developed avascular necrosis of his left hip as a result of prolonged steroid therapy. Physical examination and radiography revealed signs consistent with diffuse idiopathic skeletal hyperostosis (DISH), including a C7–T1 osteophyte causing severe tracheal compression. The patient underwent C6–T1 anterior discectomy and fusion, and the compressive osteophyte was removed, which completely resolved his “asthma.” Postoperative pulmonary function tests showed normalization of his FEV1/FVC ratio, and there was no airway reactivity on methacholine challenge. DISH is a systemic, noninflammatory condition characterized by ossification of spinal entheses, and it can present with respiratory disturbances due to airway compression by anterior cervical osteophytes. The authors present, to the best of their knowledge, the first documented case of asthma as a presentation of DISH.


2021 ◽  
Vol 14 (5) ◽  
pp. e240647
Author(s):  
Blair Wallace ◽  
Daniel Edwardes ◽  
Christian Subbe ◽  
Muhammed Murtaza

A 40-year-old patient was admitted through the acute medical take with pleuritic chest pain and rigours. He had a medical history of opiate dependence and was receiving 60 mg of methadone once daily. He was diagnosed with a community-acquired pneumonia and treated with amoxicillin and clarithromycin. After administration of only two concomitant doses of methadone and oral clarithromycin, he developed an opioid toxidrome with type-2 respiratory failure, a decreased level of consciousness and pinpoint pupils. The patient was treated with naloxone and his symptoms improved. Retrospectively, it was suspected that an interaction between clarithromycin and methadone might have contributed to the toxidrome. Respiratory failure has not been previously prescribed for this combination of medication and is of high importance for physicians and pharmacists around the world.


2021 ◽  
pp. 014556132110060
Author(s):  
Jérôme R. Lechien ◽  
Rémi Hervochon ◽  
Stéphane Hans

A 42-year-old man was referred for a week history of severe dysphagia, odynophagia, fever (39 °C), fatigue, abdominal pain, pharyngeal swelling, and multiple neck lymphadenopathies. The medical history reported a mild form of COVID-19 one month ago. The biology reported an unspecified inflammatory syndrome. The patient developed peritonitis, myocarditis, and hepatitis. A myocardium biopsy was performed. A diagnosis of Kawasaki-like disease (KLD) was performed. The occurrence of KLD in adults is rare but has to be known by otolaryngologists regarding the otolaryngological clinical presentation that may precede the multiple organ failure.


Biomedicines ◽  
2021 ◽  
Vol 9 (6) ◽  
pp. 614
Author(s):  
Pasquale Ambrosino ◽  
Antonio Molino ◽  
Ilenia Calcaterra ◽  
Roberto Formisano ◽  
Silvia Stufano ◽  
...  

Background: Growing evidence points to a key role of endothelial dysfunction in the pathogenesis of COVID-19. In this study, we evaluated changes in endothelium-dependent flow-mediated dilation (FMD) in a cohort of convalescent COVID-19 patients undergoing pulmonary rehabilitation (PR). Methods: After swab test negativization, convalescent COVID-19 patients referring to a post-acute care facility for PR were consecutively screened for inclusion. Study procedures were performed at the time of hospitalization and discharge. Results: We enrolled 82 convalescent COVID-19 patients (85.4% males, mean age 60.4 years). After PR, a significant improvement in most pulmonary function tests and exercise capacity was documented. FMD changed from 2.48% ± 2.01 to 4.24% ± 2.81 (p < 0.001), corresponding to a 70.9% increase. Significantly higher changes in FMD were found in patients without a history of vascular events as compared to those with (+2.04% ± 2.30 vs. +0.61% ± 1.83, p = 0.013). Values of forced expiratory volume in 1 s (FEV1%), forced vital capacity (FVC%) and diffusion capacity for carbon monoxide (DLCO%) significantly and directly correlated with FMD both at baseline and after PR. Patients with normal FEV1% (≥80% predicted) during the overall study period or those normalizing FEV1% after PR showed a more significant FMD change as compared to patients with persistently impaired FEV1% (<80% predicted) (p for trend = 0.029). This finding was confirmed in a multivariate analysis. Conclusions: Clinically evaluated endothelial function improves after PR in convalescent COVID-19 patients. A direct and persistent association between the severity of pulmonary and vascular disease can be hypothesized. Endothelial function testing may be useful in the follow-up of convalescent COVID-19 patients.


2021 ◽  
pp. 17-18
Author(s):  
Tejasvini Chandra ◽  
Perwez Khan ◽  
Lubna Khan ◽  
Anshika Gupta

We report bilateral proptosis as the initial presentation of Acute Myeloid Leukemia (AML) in a child. An Eight year child presented with a history of painless proptosis in the both eyes within 10 days. Radiological investigation (CT scan) showed inltration of orbit with the metastatic tumour cell. AML was diagnosed with complete blood count, General Blood Picture (GBP) and bone marrow biopsy. The presumptive diagnosis of leukemic inltration of the orbit is made. We report this case as AML can rarely present in child as a bilateral proptosis due to leukemic inltration. Urgent treatment modality for this rare condition is radiation.


2019 ◽  
Vol 9 (1) ◽  
pp. 96
Author(s):  
Yoshimasa Hachisu ◽  
Yasuhiko Koga ◽  
Shu Kasama ◽  
Kyoichi Kaira ◽  
Masakiyo Yatomi ◽  
...  

Immune reconstitution inflammatory syndrome (IRIS) is an immune reaction that occurs along with the recovery of the patient’s immunity. Tuberculosis-related IRIS (TB-IRIS) upon tumor necrosis factor (TNF)-α inhibitor treatment has been reported in non-human immunodeficiency virus (HIV) patients. However, the importance of biological treatment, as a risk factor of IRIS, has not yet been established. In this study, we examined TB-IRIS in non-HIV patients to explore the role of TNF-α inhibitor treatment. Out of 188 patients with pulmonary TB, seven patients had IRIS. We examined univariate logistic and multivariate analysis to elucidate risk factors of TB-IRIS. Univariate analysis indicated that usage of immunosuppressive drugs, TNF-α inhibitors, and history of food or drug allergy were significantly related with TB-IRIS. On initial treatment, the values of serological markers such as serum albumin and serum calcium were significantly related with TB-IRIS. There was a higher mortality rate in patients with TB-IRIS. Furthermore, multivariate analysis revealed that usage of TNF-α inhibitors, history of allergy, and serum hypercalcemia were related to TB-IRIS. Usage of TNF-α inhibitors, history of allergy, and serum hypercalcemia may be independent predictors of TB-IRIS in non-HIV patients. Since higher mortality has been reported for TB-IRIS, we should pay attention to TB patients with these risk factors.


2012 ◽  
Vol 54 (4) ◽  
pp. 231-233 ◽  
Author(s):  
Walter de Araujo Eyer-Silva ◽  
Maria Cecília da Fonseca Salgado ◽  
Jorge Francisco da Cunha Pinto ◽  
Fernando Raphael de Almeida Ferry ◽  
Rogério Neves-Motta ◽  
...  

Immune reconstitution inflammatory syndrome (IRIS) in HIV-infected subjects initiating antiretroviral therapy most commonly involves new or worsening manifestations of previously subclinical or overt infectious diseases. Reports of non-infectious IRIS are much less common but represent important diagnostic and treatment challenges. We report on a 34-year-old HIV-infected male patient with no history of gout who developed acute gouty arthritis in a single joint one month after initiating highly active antiretroviral therapy.


Sign in / Sign up

Export Citation Format

Share Document