usual interstitial pneumonia :: Article CreatorAnalyzing Histopathological, Radiological, And Clinical Features In Patients With FPF
The following is a summary of "Clinical, radiological and histopathological features of patients with familial pulmonary fibrosis," published in the June 2024 issue of Pulmonology by Jaula et al.
Familial pulmonary fibrosis (FPF) is characterized by pulmonary fibrosis affecting at least two biological relatives, presenting a diverse array of clinical features among affected individuals. This study aimed to comprehensively characterize the demographic and clinical profiles of patients with FPF, reassess high-resolution computed tomography (HRCT) scans, evaluate the histopathology of surgical lung biopsies, assess survival outcomes, and examine the applicability of risk prediction models specific to this patient population. The researchers conducted a retrospective cohort study utilizing data from Oulu University Hospital and Oulaskangas District Hospital, encompassing 68 patients diagnosed with pulmonary fibrosis (ICD 10-code J84.X) between January 1, 2000, and January 11, 2023, who also reported having at least one relative with the same condition.
Clinical information was extracted from hospital medical records, and both HRCT scans and histological specimens were re-evaluated. Among the cohort, 37 patients (54.4%) were male and 31 (45.6%) female, with mean ages of 68.6 years for women and 61.7 years for men (p = 0.003). Notably, 37 patients (54.4%) were nonsmokers. The predominant radiological pattern identified was usual interstitial pneumonia (UIP), observed in 51 patients (75.0%), followed by unclassifiable patterns (8 patients, 11.8%) and nonspecific interstitial pneumonia (NSIP) in 3 patients (4.4%). Pleuroparenchymal fibroelastosis (PPFE) was noted in 13.2% of the cases as a singular or combined pattern.
According to the 2022 guidelines for idiopathic pulmonary fibrosis (IPF), patients were classified as UIP (31 patients, 45.6%), probable UIP (20 patients, 29.4%), indeterminate for UIP (7 patients, 10.3%), or having alternative diagnoses (10 patients, 14.7%). Histopathological analysis revealed similar findings, with UIP observed in 7 patients (41.2%). Genetic variants were detected in 9 patients, including telomerase reverse transcriptase (TERT) in 6 cases, telomerase RNA component (TERC) in 2, and regulator of telomere elongation helicase 1 (RTEL1) in 1 case. The overall survival analysis indicated that 29 patients (approximately 42.6%) died or underwent lung transplantation (5 patients), with a median survival time of 39.9 months.
The composite physiology index (CPI) and the gender-age-physiology index (GAP) stage I were statistically significant predictors of survival (p < 0.001). In conclusion, the study corroborates previous findings, highlighting the diverse radiological and histopathological patterns in FPF and the presence of unique combinations of these features.
Source: respiratory-research.Biomedcentral.Com/articles/10.1186/s12931-024-02864-5
Pulmonary Fibrosis/Interstitial Lung Disease
Mission
The Program for Pulmonary Fibrosis and Interstitial Lung Disease's mission is to provide state of the art evaluation and treatment for patients with interstitial lung diseases (ILD). The University of Alabama at Birmingham ILD program is designated as a Pulmonary Fibrosis (PF) Foundation Care Center network site.
Objectives
To provide exceptional care to all of our patients To maintain outstanding collaboration and communication with our colleagues to provide best possible care for our patients To lead efforts in scientific discovery that informs the development of new and effective therapies for interstitial lung diseases, particularly idiopathic pulmonary fibrosis (IPF) To better understand disease mechanisms through translational research To reach out to the community aiming to enhance ILD awareness and help improve the standards for evaluation, treatment and follow-up of patients About Our Practice
Pulmonary fibrosis or interstitial lung diseases are chronic, progressive, lung disease characterized by disabling shortness of breath, declining exercise capacity and extensive formation of scar tissue (fibrosis) in the lung. There are several different types/causes of pulmonary fibrosis and are difficult to diagnose and treat. This program exists to better understand such illnesses, train physicians to more completely treat such illnesses, and to drive the creation of superior treatment options.
Our entire team at the ILD clinic is dedicated to improving the lives of our patients with ILD through patient care, education and research. Our multidisciplinary team combines the skills of pulmonologists, thoracic radiologists and thoracic pathologists, all of whom have specific expertise in ILD and meet weekly to discuss new patients evaluated in ILD clinic. The team also works closely with subspecialists, including the rheumatologists and lung transplant team.
TeamDr. Tejaswini Kulkarni, MD, MPH, Interstitial Lung Disease Program DirectorDr. Tracy Luckhardt, MDDr. Maria del Pilar Acosta Lara, MDDr. Kevin Dsouza, MDDr. Chao He, MD, PhDDr. Carla Copeland, MDLanier O'Hare, CRNPTonja Meadows, Nurse Manager
Pulmonary Fibrosis/Interstitial Lung Disease Program Contact Information
UAB Interstitial Lung Disease ProgramTHT 422, 1900 University BoulevardBirmingham, AL 35294-0006Phone: (205) 934-7557Fax: (205) 934-6229
Contacting the Program for Pulmonary Fibrosis and Interstitial Lung Disease
To refer a patient to this program, please contact our office administrator at (205) 934-7557 from 8 AM-5 PM, CT, Monday-Friday. In order to expedite the scheduling and the evaluation process, please forward the following documents to our office as soon as possible:
1. Last clinic note or referral letter2. Pulmonary function tests (PFTs)3. Chest X-rays and CT scans - upload films to a CD and attach reports4. Lung biopsy slides and reports, if available.
After the appointment is scheduled, your patient will receive a package containing a schedule of tests and appointments, along with the "UAB ILD Program New Patient Questionnaire," maps, and information on lodging in the Kirklin Clinic area. After the clinic visit and once all data has been reviewed, including any prior biopsies, you will receive either a letter from one of our physicians or a copy the patient's clinic visit note.
Clinical Trials
The UAB ILD Center offers clinical trial enrollment for patients with idiopathic pulmonary fibrosis and other forms of diffuse parenchymal lung disease as part of a multidisciplinary and integrated approach to treatment. Please call Ms. Melessia Wells at 205- 975-9332 for more information.
Mary Beth Scholand, MD
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Excellent pulmonologists. I highly recommend her to anyone who has interstitial lung disease.
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Dr. Mary Beth Scholand has always had the utmost concern for my health and has helped me to proceed in understanding how to navigate through meds and treatments. She continues to make ongoing care clear and listens to my concerns and questions. I have felt very blessed to be her patient.
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Dr Scholand has always been great and always very helpful.
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Dr. Scholand and I have made a great team working together in my care. I've been with her for 14 years now. The last few years she has been out of network for me but she is the only doctor I'm willing to pay for.
June 15, 2024
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Mary Beth is a master pulmonologist. She is very knowledgeable along with being kind and caring. She goes the 2nd mile!
June 15, 2024
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Dr. Scholand is an above excellent physician. She is caring, calming, knowledgeable and proactive. She has excellent diagnostic skills with a bedside manner that is outstanding and which should be taught to future doctors as a class in med school.
June 13, 2024
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Without question, I felt that she would get to the bottom of my lung problem and find an effective treatment.
June 07, 2024
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Dr. Scholand ALWAYS treats me with respect and in a warm manner. Despite my personal health issue, I enjoy seeing her and am confident she's doing all that's possible for me.
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May 15, 2024
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Does not listen all that well. Not given any info on medication that could allow the progression
May 09, 2024
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Exceptional doctor as shown by her professional and knowledgeable treatment of her patients, including me. I am very grateful I was able to get approved to be seen by her and become a patient.
May 09, 2024
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Dr. Scholand is great and I always appreciate how she includes me in decisions about my care, and truly listens to me.
May 08, 2024
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An excellent and very experienced physician, very bright and helpful with all clinical issues discussed.
April 27, 2024
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Knowledgeable, caring, expert
April 26, 2024
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Dr. Scholand is simply the very best pulmonologist. She has helped me so much and always goes the extra mile for me. I would highly recommend her and her staff.
April 18, 2024
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Dr. Scholand is such a caring doctor. She always goes the extra mile.
March 30, 2024
UH HOSPITALS AND CLINICS
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She is very knowledgeable on my lung disease.
March 30, 2024
FARMINGTON HEALTH CENTER
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The absolute best pulmonary physician
March 27, 2024
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Dr. Scholand continues to show great concern and compassion for the well being of my health. She is very competent and knowledgeable!
March 21, 2024
SUGAR HOUSE HEALTH CENTER
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She shows a great deal of compassion and has always provides me with great care and listens to everything I bring up. I could not expect better care from anyone else.
March 20, 2024
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Dr. Scholand is very knowledgeable in her field but still talks to me like I'm a friend. She asks great questions and involves me in my decisions about my care going forward. I am impressed with how well she listens to my concerns and recommends the best care for my individual needs. I feel valued and appreciate her suggestions because they are always beneficial to my health. She is amazing!
March 20, 2024
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Dr Scholand is very patient, she take time to answer my question and explain my options. Highly recommend her!
March 20, 2024
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Dr. Scholand is amazing! She takes her patients health seriously. She listens, looks at things from different angles and truly cares for her patients.
March 16, 2024
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If you have respiratory issues I highly recommend Dr Mary Beth Scholand. She is very thorough and wants to find you relief for your symptoms. So easy to talk to.
March 16, 2024
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Have worked with Mary Beth for a number of years. Have a good working relationship.
March 14, 2024
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Dr. Scholand always explains my tests and my needs to ensure my future good health. I have complete trust in her.
March 08, 2024
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DR Scholand has been so helpful and understanding of my health needs and situation. I'm very grateful to have her as my pulmonary provider.
February 16, 2024
UH HOSPITALS AND CLINICS
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She is highly knowledgeable, kind and caring. I am very happy I am under her care.
February 15, 2024
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★★★★★
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SUGAR HOUSE HEALTH CENTER
★★★★★
Doctor Scholand is very professional, knowledgable, and caring.
February 12, 2024
UH HOSPITALS AND CLINICS
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She is amazing and very compassionate.
February 09, 2024
UH HOSPITALS AND CLINICS
★★★★★
She is just an amazing Dr.
February 08, 2024
SOUTH JORDAN HEALTH CENTER
★★★★★
Dr Scholand makes me feel very cared for in my health, she suggests options fee me and she is keeping me healthy, I wouldn¿t want to go anywhere else!
January 31, 2024
SUGAR HOUSE HEALTH CENTER
★★★★★
I would recommend Dr. Scholand. She is caring and compassionate. She cares about her patients. She listens and gets all the information to provide the best care.
January 29, 2024
FARMINGTON HEALTH CENTER
★★★★★
She is very knowledgeable and cares about my well being.
January 27, 2024
SUGAR HOUSE HEALTH CENTER
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She is wonderful, I really like she is my doctor
January 27, 2024
UH HOSPITALS AND CLINICS
★★★★★
She¿s the best! I love her?I travel 430 miles ish to see her!
January 27, 2024
SUGAR HOUSE HEALTH CENTER
★★★★★
She is very knowledgeable about different forms of Interstitial Lung Disease.
January 26, 2024
SUGAR HOUSE HEALTH CENTER
★★★★★
Showed an excellent and comprehensive understanding of the medical circumstances and condition, made very clear recommendations.
January 17, 2024
SUGAR HOUSE HEALTH CENTER
★★★★★
January 10, 2024
SUGAR HOUSE HEALTH CENTER
★★★★★
Dr. Scholand is always easy to talk with and has the ability to explain in a way that is understood and builds confidence in the doctor and patient relationship.
January 02, 2024
FARMINGTON HEALTH CENTER
★★★★★
Dr. Scholand is the best part of UofU - excellent and very caring. The rest of UofU is very mismanaged.
December 16, 2023
SUGAR HOUSE HEALTH CENTER
★★★★★
I highly recommend Dr. Scholand to anyone looking for pulmonary care.
December 03, 2023
FARMINGTON HEALTH CENTER
★★★★★
December 02, 2023
FARMINGTON HEALTH CENTER
★★★★★
Dr. Scholand is by far the best doctor I have ever worked with ever!
December 01, 2023
UH HOSPITALS AND CLINICS
★★★★★
She is amazing and very caring. I never felt like she was rushing g or on a hurry.
November 29, 2023
SUGAR HOUSE HEALTH CENTER
★★★★★
Dr. Scholand is friendly, knowledgeable and thorough.
November 29, 2023
SUGAR HOUSE HEALTH CENTER
★★★★★
She is the best! She is really caring and knows my condition thoroughly. I honestly believe she does everything in her power to listen and explain and teach me and help me in my health journey.
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