Professor Stein, Rheumatologist, University of Florida, is well known to Japan and he has kindly commented on January's case. I would normally publish such comments as additions to the published cases but I think that Prof Stein's comments being so important deserve their very own spot on the blog.
Here are Prof Stein's comments below:
1. Pt's response to PSL was dramatic with decrease in her pains and increase in her activity level.
2. The PSL dramatic response represents a theraputic diagnosis, most consistent with PMR/GCA.
3. The American College of Rheumatology list 5 criteria for the diagnosis of Giant Cell Arteritis: 1) age of onset >/ 50 years, 2) New headache, 3) Temporal artery abnormality, 4) Elevated ESR and 5) Abnormal artery biopsy. The presence of any 3 has a sensitivity of 93% and a specificity of 91%.
4. This patient had 3 of these criteria in addition to the dramatic response to PSL.
5. The neuropathy the patient had has been rarely described in GCA as well as rare cases of associated nephritis
6. There is no evidence for a primary nephropathy in the patient, none for MPA; P-ANCA is non-specific inflammatory marker.
7. The case details omitted her now-presumed very limited pre-PSL daily activity level
8. Most of the suggested diagnostic tests were unnecessary and only added to her hospital costs; every patient considered for long term PSL must have a Tb evaluation.
9. In my experience Japanese physicians are not well informed about PMR/GCA; there is a great need for more rheumatologists in Japan.
I would like to comment as well.
Tuberculosis evaluation is very important in Japan. The rate of new Tb cases in Japan (a few years ago) was about 1 in 5,000 population compared to the UK which is about 1 in 8,000 new cases. The levels have apparently been decreasing in Japan whereas in the UK, they had been increasing but are now somewhat more stable. The reason for the rise in the UK is likely to be due to the large immigrant population. Japan has a more homogeneous population than the UK and fewer immigrants, although the level of Tb appears to be higher. I am uncertain why there is a higher level of Tb infection in Japan especially when there is an effective health care system in place. Perhaps others would like to comment?
Hence, as Prof Stein has mentioned, ruling out Tb prior to starting the steroids is a must. It must be done properly. Chest radiography, sputum microscopy and culture. Checking the PPD skin test is useful because a positive reaction means antibiotic therapy. Other tests can be done but which have various problems with specificity and sensitivity with attendant higher cost implications which include mycobacterial PCR and interferon testing.
Prof Stein also mentioned about over testing in this patient. He is correct. With a patient such as this, doppler ultrasonography of the temporal arteries and subsequent urgent biopsy would be necessary. Ensuring that Tb investigations were under way, steroids would need to be started urgently to avoid potential blindness if giant cell arteritis was really suspected. If Tb was also suspected e.g. cough, weight loss, sputum and CXR consistent with the diagnosis, many physicians would also commence anti-Tb therapy whilst awaiting the confirmatory Tb results.
Moreover, waiting for a biopsy result prior to starting steroid therapy is not an option in a case where GCA is high up in the differential diagnosis. In fact, steroids are advocated to be started before the biopsy if there is any potential for delay in obtaining a specimen. There is often a concern about being 100% correct about the diagnosis before starting treatments which, if delayed, can have serious adverse consequences on patients. Remember that delaying starting steroids in GCA can result in blindness. It is not something just written in the textbooks -- I have seen such a case.
Start Treatment as Soon As Deemed Clinically Necessary
Never delay life saving / organ saving treatments for fear of not wanting to be wrong. It is acceptable to give a patient with suspected heart failure the additional steroids and beta-stimulant therapy if they have obvious wheeze especially if they have a history of smoking or asthma and a physical examination consistent for both CHF and COPD. Don't just put it down to the cardiac wheeze of CHF as you will more often be wrong than right. Sometimes, patients have heart failure and COPD together. It comes down to history, physical examination, basic lab tests and basic radiology.
In another example, it is necessary to give broadspectrum antibiotics in patients with symptoms and signs of sepsis despite the lab data showing no rise in the CRP and absence of fever. Patients don't write the textbooks and the absence of some features does not preclude the condition existing. I know of one such case where a junior doctor did not appreciate the patient's low blood pressure and absence of fever despite the history of recurrent UTIs. The patient clearly had features of sepsis but the CRP was normal. However, blood cultures grew gram negative bacilli. Sepsis in the elderly can sometimes trick the doctor as it can present with so few signs, and is often a Baptism of Fire for the junior doctor.
Don't just rely on lab studies, they can sometimes be misleading. Put the pieces of the puzzle together to make the likeliest picture. Treat the serious problems first. Treatment should be based on what is likely and what cannot be immediately excluded quickly. Starting life saving treatment should be immediate and not delayed by the doctor wanting to be 100% right with the diagnosis e.g. suspected PE but unconfirmed by CT -- start heparin as soon as the diagnosis is strongly considered. Remember, the patient comes first and not the doctor's pride.
We cannot be 100% right all of the time. No one will think bad of you for treating all potential conditions that can cause the presenting symptoms and signs etc. It is a matter of protecting the patient from further harm from their condition.
The Balance of Tests Versus Treatments
It can be difficult to get the balance right between instituting tests and starting treatments. Over investigating patients does increase costs for the patient and at the same time, it seems to give physicians the confidence that certain diseases do not exist. I only wish the latter was true, but it is not. Not all tests are 100% accurate and in order to understand the investigation of a disease one needs to understand the limitation of the various lab studies and not over rely on them as is commonly done. In my experience, doctors often order a battery of tests without even considering why the tests are performed to use the 'labo' as a 'screening tool'. In my opinion, this is completely unnecessary, a waste of resources and in some cases it does not add to the identification of the problem. Tests should be ordered depending on what is considered to be wrong with the patient rather than what is done as a battery of tests.
For example, C-reactive protein is useful in only a minority of conditions such as some connective tissue diseases but in other respects it is unnecessary and expensive. I do not advocate its routine measurement. The measurement of LDH is again wasteful and lacks sensitivity and specificity. Yes, it can be measured in lymphoma where is can sometimes be very high. However, many, many other conditions also elevate the LDH level and it is somewhat like the CRP as it does not add to your diagnostic plan. Funnily, I have often heard of doctors saying ' Follow the CRP ' or ' Follow the CT '. I prefer to follow the patient.
If the diagnosis is still not clear after receiving back the information from the first set of tests, then proceeding with other specific tests can be justified. However, performing tests that simply reconfirm what you already know is pointless and wasteful e.g. chest CT scanning when a pneumonia has already been confirmed by history, physical and chest radiography. To say that it gives detailed images is correct but it DOES NOT usually add anything to diagnosis or treatment. Of course, if after a few days of treatment the fever is still swinging or the pneumonia clears to leave a mass then tumour or abscess can be investigated by CT. However, most pneumonias do not require a chest CT to confirm the diagnosis. History should guide you e.g. previous Tb, unsafe sexual practises, heavy smoker etc. Without history, we are lost and end up ordering the 'pan-man scan'.
In actual fact, a good history and thorough physical exam can tell you if there is pneumonia or effusion. Many UK GPs diagnose pneumonia and do not do blood tests (unless necessary) and may only send the patient to the hospital for a chest Xray (if deemed necessary). They start treatment based on the history and physical. Only the sick patients get referred for inpatient treatment.
Cultural Medical Practise
When we consider the practise of medicine we must also consider the cultural practises rather than just a rigid template of 'this is the way we do it in this country', because it may not be translatable in another culture.
For example, in Japan, there is much choice of where you can go to see a doctor. Basically, you can turn up in any hospital you like and see any doctor you like who specialises in the condition you think you have. This is not the case in the UK NHS system whereby you are usually allocated a GP servicing your local community and you are referred to the local hospital for outpatient consultations by your GP. You cannot just turn up in the outpatients. Of course, ER (A&E) attendances do not require an appointment in the UK. However, choice is lacking in the UK system.
Japanese hospitals are a mixture of public and private compared to the British which is predominantly public.
Because patients have so much choice in Japan, the adverse impact is that some ask for many tests and indeed, some expect the battery of blood tests, CT and MRI even if they are not always medically justified. Sometimes, the doctor can feel pressurised into performing unnecessary tests. Of course, patients are quite rightly concerned about potential types of illness but it is the role of the doctor to be the gate-keeper to stop patients having unnecessary annual CT scans and serum rhubarb evaluations to rule out the rarest of conditions. Remember, common things are common. Rule out the common things before trying to identify the zebra amongst the drove of horses.
Luckily, with the advent of the DPC system, which limits the amount paid for a particular condition, it will help to reduce overall costs to the Japanese Government for health care and also to the patient and their family who need to pay 30% of the overall medical costs. Therefore, over investigating patients is no longer a realistic option and doctors need to become more reliant on clinical skill with history taking and physical examination techniques. This can only be done through effective training at university and in the post-graduate residency training schemes.
Need for More Rheumatologists
Prof Stein also quite rightly says there should be more rheumatologists in Japan. As with palliative care and oncology specialists, rheumatologists are few and far between.
Many patients with rheumatology problems are seen by orthopedic doctors. Training as a rheumatologist is a medical sub-specialty rather than a surgical specialty. Such study requires an in-depth knowledge about systemic disease requiring many years of training in internal medicine. Hence, although orthopedic doctors do a sterling job to try and treat patients with connective tissue disease, they should be really seen by the rheumatology specialists for fine tuning of the diagnosis and therapeutics.
Unfortunately, drugs tend to be used at lower dosages in Japan. For example, methotrexate is commonly given at 5mg per week for rheumatoid arthritis whereas most UK / American rheumatologists would titrate the dose to the maximum tolerated to relieve the symptoms and destructive changes e.g. 15mg/week. It is not correct to think that just because a patient has a connective tissue disease e.g. rheumatoid arthritis, that they should expect to develop debilitating disease because that is the expected natural history of the condition. If DMARDs are going to be used, then they should be used according to evidence rather than eminence.
There are many differences between the Japanese system and the UK-USA systems.
It is clear to me that doctors need to have more skill in history taking and physical examination. More urgency needs to be taken over starting life / organ saving therapies rather than trying to be 100% correct at the outset with the attendant wait resulting in potential and real detriment of the patient. Patient's should not be over investigated and tests should be based on what is considered to be wrong with the patient and what serious diseases need to be ruled out quickly e.g. Tb. Treatments should be provided based on relevant evidence from history, physical, labs and radiology etc, to cover the various potential serious conditions e.g. heparin in suspected (unconfirmed) PE.
I come back to the use of the problem list and grouping technique, as taught by Prof Stein in his Problem Oriented System, in order to decide what are the likely causes of the various symtpoms and signs. By using this process, junior doctors can understand the various problems which aide them to make a diagnostic and therapeutic plan for patients.
Many thanks to Professor Stein for his very necessary comments.