A man was diagnosed to have myositis ossificans progressiva at the age of 20 years. He died five years later. What is the most probable cause of his death-
**Myositis Ossificans Progressiva (Fibrodysplasia Ossificans Progressiva, FOP)**
**Core Concept**
Myositis ossificans progressiva, also known as fibrodysplasia ossificans progressiva (FOP), is a rare genetic disorder characterized by the progressive ossification of soft tissues, including muscles, tendons, and ligaments. This leads to the formation of abnormal bone tissue, resulting in loss of mobility and eventual disability.
**Why the Correct Answer is Right**
The most probable cause of death in a patient with FOP is a complication related to respiratory or cardiac involvement. In FOP, the progressive ossification of the chest wall and diaphragm can lead to restrictive lung disease, while the involvement of the heart can cause arrhythmias or cardiac failure. The patient's death is likely due to respiratory or cardiac failure. The pathophysiology of FOP involves the activation of the ACVR1 gene, leading to the formation of heterotopic ossification, which can cause respiratory and cardiac complications.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is not relevant to the most probable cause of death in FOP patients.
**Option B:** While infections can be a complication in patients with FOP, they are not the most probable cause of death.
**Option C:** Although FOP patients can experience musculoskeletal pain and joint stiffness, these symptoms are not typically life-threatening.
**Option D:** This option is not a known complication of FOP.
**Clinical Pearl / High-Yield Fact**
FOP is a rare genetic disorder with an estimated incidence of 1 in 2 million births. It is characterized by the presence of a specific mutation in the ACVR1 gene, which leads to the formation of heterotopic ossification.
**Correct Answer:** D.