This morning I looked at the map of the day
And said to myself, “This is the way! This is the way I will go;
Thus shall I range on the roads of achievement,
The way is so clear—it shall all be a joy on the lines marked out.”
And then as I went came a place that was strange,—
’Twas a place not down on the map!
And I stumbled and fell and lay in the weeds,
And looked on the day with rue.
Experience – Carl Sandburg
The first inclination that something nasty was happening in my body was pain – both upper arms and radiating across the top of my chest. It was so severe that it was impossible to ignore, but my concerns were quickly brushed away by the trainee doctor who had seen me urgently at my GP surgery. He thought it was merely the flu. I wasn’t impressed. As an emergency doctor with more than 30 years experience I would never have seen a doctor because of the flu!
A few days later I went to the Emergency Department to have some more tests. My initial liver function tests had been very abnormal. While there, it was discovered that my CK was 24,000. Four weeks and a multitude of investigations later, I had a diagnosis: Seronegative Immune Mediated Necrotising Myositis.
By this stage I was so weak that I was unable to get out of bed or even brush my hair – but amazingly I could still walk! Over the next eight weeks my symptoms slightly improved with prednisone and Myfortic. Shortly after this IVIGs resulted in rapid improvement with onset of full movement 24 hours later. So far, everything seemed reasonably straightforward. But six months into treatment my rheumatologist started voicing concern and doubt over my symptoms. They didn’t make sense to him.
I would have a few days of weakness prior to each infusion of IVIGs. This would quickly resolve within 24-48 hours. My rheumatologist would look at me confusedly and say that we needed to start decreasing my IVIG dose soon. I would look at him in amazement. How could he say that when I got so weak before them? Decreasing the dose would only increase my periods of weakness. I proved this theory by talking him into increasing my dose! I had gained almost 10 kg and hoped that an extra 10 grams of IVIGs would decrease my days of weakness every month from 6 down to 2. It did.
But after twelve months of treatment, suddenly and unexpectedly, I relapsed completely. I had markedly increased weakness before my IVIGs in June, and only moderate improvement afterwards. I hadn’t been this weak since starting IVIGs. I wracked my brain for a possible cause. I had had my flu and COVID boosters two weeks earlier, but as a rational, nonconspiratorial theory doctor, I refused to consider this a contributing cause. This was definitely correlation and not causation.
Maybe the IVIGs were the problem…for some reason inactivated by poor storage. This seemed unlikely. The third option was my decreased dose of Myfortic, done in the hope that my body would be able to fight off a fungal lung infection. I looked at my calendar and counted back…11 weeks. That was probably the culprit. I had assumed that my myositis was being perfectly controlled by my IVIGs and had had no thought of relapse. I was wrong.
I texted my rheumatologist about the recurrence of my weakness and he replied that it was unlikely that it was due to my myositis! My CK was normal. Instead he wanted to continue to decrease my immunosuppressants. I sighed exasperatedly and messaged him back, sarcastically asking if he thought I should see a psychiatrist? His only reply was that as a doctor myself, I should know that he just couldn’t take my word for my symptoms. I wondered how you could possibly be a doctor without believing your patients’ stories? I’ve always found that it is the story that gives the diagnosis….examination of the patient and investigations done purely to prove or disprove the original hypothesis.
Two weeks later, I had the onset of rapidly increasing pain in my upper arms. I did not message my rheumatologist this time. I still hadn’t recovered from the mental trauma of having my weakness summarily fobbed off. He would be far less impressed with pain than he was with weakness. I was due to have surgery for an Aspergillus lung abscess at the end of the month. It was probably better to have a partially functional immune system than a fully functional set of muscles. This fight could wait until after my surgery. Maybe everything would come good after my next dose of IVIGs.
It did not. I did get better, but it didn’t last for long. I had five days of being able to walk normally before my severe weakness returned. At the end of that month my infectious diseases specialist cleared me to increase my dose of Myfortic. He had made the original decision to decrease it. I discussed this with my GP and told her she had to message my rheumatologist about it. After all, he had said he couldn’t take my word for my symptoms. She had seen me every two weeks and had documented my weakness meticulously.
Unfortunately, she forgot. She had had a medical student that day and had been distracted. Maybe it was better this way… it was probably easier to ask for forgiveness than for permission. I was prone to ignoring stupid advice anyway, so I was going to increase it no matter what he said.
Two months later, at my next appointment, my rheumatologist appeared annoyed but patiently listened to my story. He could follow my logic even if he didn’t understand the pathophysiology of the disease process. I could be weak due to muscle destruction, but it shouldn’t fluctuate so quickly. I could have pain due to muscle inflammation and destruction, but then my CK should be elevated. My increased dose of Myfortic should have only taken one month to take effect. I had now been on it for two months.
I shrugged. I’m sure there are many aspects of this weird disease that no one understands. He thought a big city rheumatologist would know more than him. I thought this was unlikely. Myositis is so rare that few specialists know very much about it. He had given me cutting-edge treatment from the very beginning. However, I could see his point. I was becoming more and more difficult as a patient…rapidly increasing in both my lived experience as well as my learned knowledge about my rare disease. Now that I was no longer incredibly unwell from my lung abscess, I had more confidence in my own decision-making ability than his.
I managed to talk him into giving me a repeat induction dose of IVIGs….two doses in four weeks…to help stabilise my active disease process. I had discussed this option with Australia’s leading myositis researcher, whom I had met at a conference, and assured him that it was easy. It was. He probably felt I was telling him what to do, but I had no choice. It had now been four months since I had been able to walk normally. We had to do something!
The day after my appointment, I was walking down the hall when I realised my hip muscles were working again. I had been on the increased dose of Myfortic for 9 weeks. I immediately went for a 500-step walk. After four months of struggling to walk at all, it felt amazing! However, later that week I had recurrence of weakness a few days before my next infusion of IVIGs.
As I reviewed the literature on the causes of weakness in myositis, it became clear that the broad view that weakness is primarily due to muscle destruction was overly simplistic. There were numerous other changes in muscle pathophysiology, many of them preceding muscle destruction. Even in a single patient, multiple mechanisms may coexist and vary with the disease phase. Some patients have more weakness than their histopathology suggests. (3)
Cytokines were produced in response to the body’s reaction to the pathogenic antibodies. There were myokines. There were changes in receptors that regulate water entering muscle cells. There were changes to how energy is produced in the cells, making them less able to function normally. Every paper ended with the conclusion that very little is known about why and how weakness develops in myositis.
Tumour Necrosis Factor may affect calcium release from the sarcoplasmic reticulum. This then results in muscle fatigue. There are many other cytokines that could also affect the muscle’s ability to function normally. These could be reversed quickly with treatment, thus accounting for my rapid improvement after starting immunoglobulins. As these cytokines and interleukins increase as my IVIGs wear off, so does my weakness.
Overall, myositis is a mystery, and it is impossible to make it simple and understandable. If a rheumatologist doesn’t understand it, what hope does even a doctor patient have? But the takeaway is that the important thing is to listen to the patient, and not to assume that if what the patient is saying doesn’t make sense, that is the fault of the patient. It is the fault of the disease!
It was obvious to me that my upper arm pain was due to my myositis, but what was the mechanism? I had to sleep flat on my back as I was unable to put any pressure on my upper arms without causing severe pain. Constant background pain meant I had to take regular Panadol and Nurofen to keep it to a dull ache. But unlike muscle function, pain is much harder for the clinician to assess. Some rheumatologists even deny that myositis causes pain at all!
That is a view that is well out of date, with several recent articles documenting pain in myositis. But with myositis being such a rare disease, they may have only seen a few patients. It is easy to assume that all patients should be the same, but in fact, our immune systems are even more different than our fingerprints. The possibility of any two patients being exactly the same is remote.
An article was published in Rheumatology titled “Pain is common in myositis and associated with disease activity.” It found that up to 50% of myositis patients have pain, particularly those with IMNM. Increasing pain was well correlated with worsening muscle weakness. As the muscle weakness improved, so did their pain scores.
Muscle inflammation resulting in destruction has long been thought to be the main cause of myositis pain. Therefore, if the CK was normal, pain was dismissed as not being due to myositis, and instead the patient was frequently given an alternate diagnosis such as fibromyalgia. However, the article mentions fasciitis as also causing pain. This does not cause a high CK, though Aldonase may be elevated.
The penny dropped. There was a rational mechanism. Inflamed fascia is less likely to appear on MRI. The pain was stabbing rather than just aching. Within a few weeks, my range of motion would decrease rapidly as my inflamed fascia became stiff and inelastic. Again, it underscored the importance of listening to the patient, not just looking at their blood tests. My blood tests were all normal, but my recurrence of symptoms was definitely due to my myositis.
An elevated CK may be helpful, but its absence does not exclude a relapse of myositis. The clinical examination is far more reliable in determining disease activity. If there is a dramatic change in the patient’s ability to raise their arms above their head, lift their legs, and raise their head off the bed, these are due to the disease, even if the CK remains in the normal range.
After my double dose of IVIGs, I got seven weeks of no weakness. My muscle strength increased rapidly, and my pain decreased to almost zero. Then my weakness and pain returned. This time however, it was much slower as well as less severe. I was always able to walk around the house, though I struggled to walk on uneven ground or hills…downhill being worse than uphill as my hip flexors struggled against the increased load. My pain was primarily muscle tenderness in my triceps, biceps, and lateral thigh muscles. I was able to sleep lying on my side, and it didn’t really limit my activity.
Over the next three months, my periods of weakness before my IVIGs slowly decreased. I was now able to walk for 30 minutes at a time with minimal muscle fatigue. My pain continued to slowly improve, even as I decreased my prednisone dose. I still struggled before my IVIGs, but my ability to move returned rapidly within 24 hours after them. After almost two years of sick leave, I made plans to return to work. But with this disease, there is no map laid out. No road is sure.
I am learning a little—never to be sure—
To be positive only with what is past,
And to peer sometimes at the things to come
As a wanderer treading the night
When the mazy stars neither point nor beckon,
And of all the roads, no road is sure.
~Experience – Carl Sandburg ~
Loelll and Lundberg wrote in their paper, Can muscle regeneration fail in chronic inflammation: a weakness in inflammatory myopathies? (3) that “These mechanisms may vary between patients and in different phases of the disease. It has been suggested that the cause of muscle weakness is loss of muscle fibres because of fibre degeneration and necrosis as a result of direct cytotoxic effects of T cells. However, there is a dissociation between the degree of histopathological changes and the degree of muscle weakness, which suggests that other mechanisms may also lead to low muscle performance. One possibility is an effect of cytokines, released from the infiltrating inflammatory cells, on muscle contractile properties including effects on Ca2+ release from the sarcoplasmic reticulum as has been demonstrated for tumour necrosis factor (TNF) and more recently also for the alarmin high-mobility group box (HMGB)1. Both TNF and HMGB1 have been detected in muscle tissue of myositis patients . Another mechanism that may contribute to the low muscle performance is loss of capillaries in muscle tissue and, as a consequence, tissue hypoxia which could have a negative effect on muscle performance. There are also signs of nonimmune mechanisms in muscle tissue of myositis patients, including endoplasmic reticulum (ER) stress, that could affect muscle performance.
References:
1. The molecular basis of skeletal muscle weakness in a mouse model of inflammatory myopathy – Arthritis Rheumatology Nov 2012
https://pmc.ncbi.nlm.nih.gov/articles/PMC3485437/
2. Idiopathic inflammatory myopathies: pathogenic mechanisms of muscle weakness – Skeletal Muscle 2013
https://link.springer.com/article/10.1186/2044-5040-3-13
3. Can muscle regeneration fail in chronic inflammation: a weakness in inflammatory myopathies? Journal of Internal Medicine Dec 2010
https://onlinelibrary.wiley.com/doi/full/10.1111/j.1365-2796.2010.02334.x
4. Idiopathic inflammatory myopathies: why do the muscles become weak? Current Opinion in Rheumatology 2001
5. Pathogenesis of Muscle Weakness in Inflammatory Myositis – Indian Journal of Rheumatology Dec 2020
https://journals.lww.com/ijru/fulltext/2020/15002/
6. Impaired muscle strength is associated with ultrastructure damage in myositis – Scientific Reports Oct 2022
https://pmc.ncbi.nlm.nih.gov/articles/PMC9586957/
7. Aquaporin-4 downregulation in dysphagic patients with idiopathic inflammatory myopathies: myofiber vulnerability and inflammation drivers – Rheumatology Dec 2025
https://academic.oup.com/rheumatology/article/64/12/6378/8237399
8. Disease spectrum of myopathies with elevated aldolase and normal creatine kinase – European Journal of Neurology Nov 2023
https://pmc.ncbi.nlm.nih.gov/articles/PMC11235866/
9. Quality of life in patients with myositis is associated with functional capacity, body composition, and disease activity-Baseline data from a randomized controlled trial – International Journal of Rheumatology Diseases 2024 Nov/Dec 2024
https://pubmed.ncbi.nlm.nih.gov/18021522/
10. Disease-specific quality indicators, outcome measures and guidelines in polymyositis and dermatomyositis – Clinical and Experimental Rheumatology Nov/Dec 2007
https://pubmed.ncbi.nlm.nih.gov/18021522/
11. Pain profile and opioid medication use in patients with idiopathic inflammatory myopathies – Rheumatology May 2022
https://academic.oup.com/rheumatology/article/62/1/264/6586791
12. Patients’ experiences of managing their rare rheumatic disease – Rheumatology Nov 2025
https://academic.oup.com/rheumatology/article/64/11/5698/8162704
13. Pain is common in myositis and associated with disease activity – Rheumatology Feb 2025
https://academic.oup.com/rheumatology/article/64/2/780/7614639#google_vignette
14. Myalgia in Patients with Dermatomyositis and Polymyositis Is Attributable to Fasciitis Rather Than Myositis: A Retrospective Study of 32 Patients who Underwent Histopathological Examinations – The Journal of Rheumatology April 2017
https://www.jrheum.org/content/44/4/482
15. Pain in autoimmune inflammatory myopathies: a scoping review – RMD Open: Rheumatic and Musculoskeletal Diseases Jan 2023
Beauty for Ashes 
Hi Jennie,
I just love this OMG so great. I wish I could put my journey side by side on paper like you did . We would be sisters from anothe mother. I have Dermatomyositis with ILD/Overlap. I would love to try and connect sometime and talk .
My Best
Karen