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Developing drugs for rare diseases presents unique challenges from a statistical perspective. These challenges may include slowly progressive diseases with unmet medical needs, poorly understood natural history, small population size, diversified phenotypes and genotypes within a disorder, and lack of appropriate surrogate endpoints to measure clinical benefits. The Real-World Evidence (RWE) Scientific Working Group of the American Statistical Association Biopharmaceutical Section has assembled a research team to assess the landscape including challenges and possible strategies to address these challenges and the role of real-world data (RWD) and RWE in rare disease drug development. This paper first reviews the current regulations by regulatory agencies worldwide and then discusses in more detail the challenges from a statistical perspective in the design, conduct, and analysis of rare disease clinical trials. After outlining an overall development pathway for rare disease drugs, corresponding strategies to address the challenges are presented. Other considerations are also discussed for generating relevant evidence for regulatory decision-making on drugs for rare diseases. The accompanying paper discusses how RWD and RWE can be used to improve the efficiency of rare disease drug development.
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http://dx.doi.org/10.1002/cpt.3631 | DOI Listing |
Genome Biol
September 2025
Center for Genomic Medicine, Cardiovascular Research Center, , Massachusetts General Hospital Simches Research Center, 185 Cambridge Street, CPZN 5.238,, Boston, MA, 02114, USA.
Background: Rare genetic variation provided by whole genome sequence datasets has been relatively less explored for its contributions to human traits. Meta-analysis of sequencing data offers advantages by integrating larger sample sizes from diverse cohorts, thereby increasing the likelihood of discovering novel insights into complex traits. Furthermore, emerging methods in genome-wide rare variant association testing further improve power and interpretability.
View Article and Find Full Text PDFBMC Cardiovasc Disord
September 2025
Department of Cardiology II (Electrophysiology), University Hospital Münster, Albert-Schweitzer-Campus 1, Münster, 48149, Germany.
While most sudden cardiac deaths are due to structural heart disease or cardiac ischemia, intoxications are rather rare and often unrecognized. Here we present a case of a 35-year-old patient who trickled cumulative 60 mg of the pure nicotine liquid. This led to cardiac arrest and ventricular fibrillation.
View Article and Find Full Text PDFBMC Neurol
September 2025
Department of Neurology, University Hospital, RWTH Aachen University, Pauwelsstrasse 30, Aachen, North Rhine-Westphalia, Germany.
Background: Cerebellar pathologies in adults can have a wide range of hereditary, acquired and sporadic-degenerative causes. Due to the frequency in daily hospital, especially intensive care, settings, electrolyte imbalances are an important, yet rare differential diagnosis. The hypomagnesemia-induced cerebellar syndrome (HiCS) constitutes a relevant disease entity with clinical and morphological variability due to a potential progression of symptoms and a promising causal treatment.
View Article and Find Full Text PDFClin Rheumatol
September 2025
The First College of Clinical Medical Science, Three Gorges University, Yichang, China.
Background: IgG4-related lung disease (IgG4-RLD) is a rare autoimmune condition. This study aims to systematically analyze the clinical characteristics of IgG4-RLD to enhance clinicians' awareness and improve patient outcomes.
Methods: This retrospective analysis investigates the clinical data of 20 patients diagnosed with IgG4-RLD at the Yichang Central People's Hospital between January 2019 and April 2025.
Pediatr Nephrol
September 2025
Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India.
Background: Kidney involvement in pediatric sarcoidosis is rare and often underrecognized, leading to diagnostic delays and treatment challenges. We report six patients with renal sarcoidosis to highlight their diverse presentations and outcomes and challenges in management.
Methods: Medical records of patients diagnosed with renal sarcoidosis during 2020-24 were reviewed.