Intravenous Monthly High-Dose Pulse Methylprednisolone vs Oral Prednisone for Therapy of Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis.

Primary CNS angiitis (PACNS) is distinguished from other vasculitides by its exclusive involvement of the CNS, generally in a multifocal manner. Lesions are typically, but not invariably, ischemic or hemorrhagic. Symptoms most frequently include headache, but also commonly involve cognitive impairment, focal neurological symptoms, and sometimes seizures. The disorder may involve medium or small-sized vessels. MRI of brain and spinal cord is usually abnormal, frequently showing cortical and subcortical infarcts. However, in the case of smaller vessel involvement, ischemic demyelination may be seen. Angiography frequently suggests the diagnosis by demonstration of “beading,” but biopsy is often necessary to firmly establish the diagnosis. Treatment is usually with high-dose pulse methylprednisolone followed by a prolonged course of oral prednisone, but the addition of other immunosuppressant medication is often necessary.

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Fungal disease in cystic fibrosis and chronic respiratory disorders

10.1093/med/9780198755388.003.0037 ◽

2017 ◽

Author(s):

Chris Kosmidis ◽

David W. Denning ◽

Eavan G. Muldoon

Keyword(s):

Cystic Fibrosis ◽

Allergic Bronchopulmonary Aspergillosis ◽

Fungal Disease ◽

Chronic Obstructive ◽

High Dose ◽

Obstructive Pulmonary Disease ◽

Chronic Respiratory Diseases ◽

Fungal Organisms ◽

Seriously Ill Patients ◽

The Impact

A range of fungal disease syndromes affect patients with chronic respiratory diseases and cystic fibrosis (CF). Invasive aspergillosis is increasingly recognized in seriously ill patients with chronic obstructive pulmonary disease, especially after high-dose steroids. Chronic pulmonary aspergillosis affects patients with pre-existing cavities or bullae, such as those with previous tuberculosis or atypical mycobacterial disease, bullous emphysema, sarcoidosis, pneumothorax, or treated lung cancer. In addition, fungi have become one of the most important trigger agents for asthma, and allergic bronchopulmonary aspergillosis may complicate up to 3.5% of cases of asthma and up to 15% of cases of CF, starting in childhood. CF patients are commonly colonized with fungal organisms, although the impact of such colonization on outcome is not clear. Aspergillus is the most common mould isolated from CF patients. Distinguishing between colonization and infection remains challenging. Candida is thought to be of no clinical significance; however, it has been associated with decline in lung function.

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A Case of Allergic Bronchopulmonary Aspergillosis (ABPA) in a Patient with a History of Cocaine Use and Tuberculosis

Case Reports in Medicine ◽

10.1155/2019/3265635 ◽

2019 ◽

Vol 2019 ◽

pp. 1-4

Author(s):

Noura Ayoubi ◽

Samuel Jalali ◽

Nikesh Kapadia

Keyword(s):

Cystic Fibrosis ◽

Weight Loss ◽

Oral Prednisone ◽

Allergic Bronchopulmonary Aspergillosis ◽

Healthcare Providers ◽

Chest Ct ◽

Cocaine Use ◽

Chronic Cocaine ◽

History Of ◽

Airway Hypersensitivity

Aspergillosis refers to a spectrum of disorders that can occur due to colonization with the Aspergillus fungus. Allergic bronchopulmonary aspergillosis (ABPA) is an airway hypersensitivity reaction to the fungus that is almost exclusively seen in patients with cystic fibrosis or asthma. Here, we present a case of ABPA in a patient with a history of chronic cocaine use and tuberculosis and no history of asthma or cystic fibrosis. The patient had presented with progressively worsening dyspnea for three months as well as a 20-pound weight loss. Diagnosis was made with an elevated IgE against Aspergillus and chest CT findings, which included bronchiectasis and tree-in-bud nodular opacities. The patient was treated with IV methylprednisolone followed by a 4-day course of oral prednisone, with significant improvement. It is our hope to make healthcare providers aware of the potential presence of ABPA in chronic cocaine users and patients with tuberculosis, both of which are not traditionally associated with this condition.

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Severe Small Airways Disease Resistant to Medical Treatment in a Child with Cystic Fibrosis

Journal of the Royal Society of Medicine ◽

10.1177/014107689608900318 ◽

1996 ◽

Vol 89 (3) ◽

pp. 172P-173P ◽

Cited By ~ 3

Author(s):

J Davies ◽

M Rosenthal ◽

A Bush

Keyword(s):

Cystic Fibrosis ◽

Medical Treatment ◽

Allergic Bronchopulmonary Aspergillosis ◽

Therapeutic Strategies ◽

High Dose ◽

Small Airways ◽

Unknown Aetiology ◽

Steroid Dependent ◽

Oral Steroids ◽

Dose Oral

At the age of 12, a child with cystic fibrosis developed severe small airways obstruction of unknown aetiology, in the absence of significant bronchiectasis. He remained resistant to medical treatment until, following an exacerbation of allergic bronchopulmonary aspergillosis 18 months later, he responded to high dose oral steroids. He now remains steroid-dependent, and suffering from multiple side-effects. Possible aetiology and further therapeutic strategies are discussed.

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Faculty Opinions recommendation of High-dose oral N-acetylcysteine, a glutathione prodrug, modulates inflammation in cystic fibrosis.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.12520.470002 ◽

2006 ◽

Author(s):

Shruti Paranjape

Keyword(s):

Cystic Fibrosis ◽

High Dose ◽

Dose Oral

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Allergic Diseases Caused by Aspergillus Species in Patients with Cystic Fibrosis

Antibiotics ◽

10.3390/antibiotics10040357 ◽

2021 ◽

Vol 10 (4) ◽

pp. 357

Author(s):

Aidan K. Curran ◽

David L. Hava

Keyword(s):

Cystic Fibrosis ◽

Fungal Infections ◽

Allergic Bronchopulmonary Aspergillosis ◽

Hyphal Growth ◽

Allergic Diseases ◽

Chronic Obstructive ◽

Obstructive Pulmonary Disease ◽

Th2 Immune Response ◽

Difficulty Breathing ◽

Oral Corticosteroids

Aspergillus spp. are spore forming molds; a subset of which are clinically relevant to humans and can cause significant morbidity and mortality. A. fumigatus causes chronic infection in patients with chronic lung disease such as asthma, chronic obstructive pulmonary disease (COPD) and cystic fibrosis (CF). In patients with CF, A. fumigatus infection can lead to allergic disease, such as allergic bronchopulmonary aspergillosis (ABPA) which is associated with high rates of hospitalizations for acute exacerbations and lower lung function. ABPA results from TH2 immune response to Aspergillus antigens produced during hyphal growth, marked by high levels of IgE and eosinophil activation. Clinically, patients with ABPA experience difficulty breathing; exacerbations of disease and are at high risk for bronchiectasis and lung fibrosis. Oral corticosteroids are used to manage aspects of the inflammatory response and antifungal agents are used to reduce fungal burden and lower the exposure to fungal antigens. As the appreciation for the severity of fungal infections has grown, new therapies have emerged that aim to improve treatment and outcomes for patients with CF.

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