Jane Austen’s Mystery Illness
Jane Austen died in 1817 at the relatively tender age of forty-one. Her last novel, Sanditon, was begun in late January of this same year but was never completed as her health began to fail severely in April and she passed away in June. For two centuries afterward, physicians, researchers, and historians have theorized about the nature of the chronic illness that led to her early death. In 1964, Sir Vincent Zachary Cope, MD, proposed her medical history suggested a diagnosis of Addison’s Disease, a widely-held theory in the decades following his biography of the young author. Subsequent historians have also advanced Hodgkins Lymphoma as a possible culprit. I would like to advance another retrospective diagnosis based on my own intimate knowledge of a very specific disorder: Relapsing Polychondritis.
Like Addison’s Disease, Relapsing Polychondritis results from the dysfunction of the body’s immune response, but instead of a failure of the adrenal glands to produce sufficient cortisol to maintain homeostasis throughout the body, Relapsing Polychondritis, or RP, results from an overactive immune response to specific autoantibodies targeting cartilage and other proteoglycan-rich tissues. RP is a very rare progressively degenerative disorder characterized by periods of remission and relapse causing consistent decline over a number of years. Affecting between one and three out of a million annually, in the early nineteenth century it was not yet recognized as a diagnosis in party with other autoimmune conditions.
Otitis Externa
The hallmark symptom of Relapsing Polychondritis is an inflamed and painful outer ear.
Jane Austen’s letters detail numerous symptoms from the age of twenty consistent with RP. Autoimmune conditions share many systemic symptoms with both Addison’s and Lymphoma, including the severe fatigue Ms. Austen experienced in increasing degrees toward the end of her life. Neither Addison’s nor Lymphoma, however, can account for the entire multitude of intermittent symptoms reported in her diary and letters throughout the course of her life. In fact, only a handful of disorders might account for one symptom in particular which Ms. Austen describes as an infection of the external ear causing great pain and accompanied by hearing loss that recovered in due course after a prescription from a Mr. Lyford.
RP has many associated symptoms and can affect any part of the body with connective tissue, but the hallmark symptom typically leading to diagnosis in the twenty-first century is the presence of inflammation of the outer ear or pinna, a rare occurrence with very few causes. The inflammation often extends into the cartilaginous ear canal and into the connective tissues around the inner ear and eustachian tubes. Sufferers may experience muffled hearing, the feeling of constant pressure as though under water, and persistent popping sounds. Swelling near the inner ear may also cause vertigo and tinnitus. Were Ms. Austen to report to a rheumatologist today the tally of her recurring symptoms, it is likely a modern-day specialist would be suspicious of RP or a similar autoimmune disorder called GPA. Given that she was able to find periods of recovered health recurring with periods of progressively deteriorating relapse, and given that the initial symptoms were not immediately fatal without immunosuppressant therapy, GPA would be ruled out.
Virtually all of Jane Austen’s decades-long symptoms may be attributable to Relapsing Polychondritis, and it is likely that her final days were triggered by the ramping immune response from the increased stress of her brother’s bankruptcy and her own diminishing hormones as she advanced toward middle age. Today, Relapsing Polychondritis still has a grim prognosis with a shortened lifespan even with cutting edge biologic immunotherapy. In Regency era England, the condition would result in an early demise even under the care of the best practitioner and Ms. Austen’s fate would have been sealed from an early age.
I am one of the rare sufferers of RP today, and Ms. Austen is an inspiring role model for me. Through two decades of ill health, she focused on a deep love for her family, gratitude for what life offered her, and a devotion to her work writing multiple timeless novels that enable her legacy to live on.
