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Microscopic polyangiitis and myasthenia gravis: the battle of Occam and Hickam
Michael V Holmes1, Debajit Sen
1Centre for Rheumatology, University College Hospital, 3rd Floor Central, 250 Euston Road, London, NW1 2PQ, UK.
Abstract:
Microscopic polyangiitis (MPA) is a pauci-immune, necrotising, small-vessel vasculitis with an incidence of 3.6 per million population that typically presents in adulthood. Myasthenia gravis (MG), the most common disorder of the neuromuscular junction is rare, with an incidence of four per million population. We present the case of an adolescent girl previously diagnosed with MPA at age 7 years who presented with breathlessness and respiratory failure aged 15 years. The respiratory symptoms were due to thymoma-MG, which was successfully treated with cholinesterase inhibitors and thymectomy. This case report illustrates that the well-established doctrines of Occam's razor and of 'common conditions occurring commonly' are not universally applicable, and that in the adolescent age group, one should still consider Hickam's dictum.
Insights
Microscopic polyangiitis (MPA) and myasthenia gravis (MG) are rare conditions. This case highlights a rare co-occurrence in an adolescent, emphasizing the need to consider uncommon diagnoses.
Area of Science:
- Rheumatology and Neurology
- Pediatric Autoimmune Diseases
Background:
- Microscopic polyangiitis (MPA) is a rare autoimmune vasculitis affecting small blood vessels, typically diagnosed in adults.
- Myasthenia gravis (MG) is a rare neuromuscular junction disorder characterized by muscle weakness.
Observation:
- A 15-year-old female, previously diagnosed with MPA at age 7, presented with severe breathlessness and respiratory failure.
- Her respiratory symptoms were attributed to a concurrent diagnosis of thymoma-associated myasthenia gravis (thymoma-MG).
Findings:
- The patient's thymoma-MG was effectively managed with cholinesterase inhibitors and surgical thymectomy.
- This case demonstrates a rare co-presentation of MPA and thymoma-MG in an adolescent.
Implications:
- Challenges the 'Occam's razor' principle in complex pediatric cases, suggesting multiple conditions can coexist.
- Highlights the importance of considering less common diagnoses (Hickam's dictum) in adolescent respiratory failure, even with a prior vasculitis diagnosis.
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