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MINI BLOG – Spondyloarthropathy Extra-Articular Symptoms

Intro

Just as a reminder… Spondyloarthritis (SpA) is an umbrella term covering auto-immune conditions affecting the Axial skeleton (the spine and sacro-illiac joints). Conditions falling under this umbrella include, Ankylosing Spondylitis and Psoriatic Arthritis. This blog will outline some of the extra-articular features associated with these conditions, it is important to recall that these are not co-morbidities which are separate conditions which can co-exist alongside the discussed condition. These will be explored in different blogs as they have a significant impact on SpA.

PLEASE REMEMBER – THIS BLOG IS NOT A REPLACEMENT FOR CLINICAL REASONING, IF YOU ARE UNSURE GET ADVICE

Just for clarity, the following percentages are the prevalence of the extra-articular feature in the lifetime of a diagnosed SpA patient.

Common extra articular features include:

Peripheral arthropathy/synovitis – 30%

Peripheral Enthesitis – 40%

Dactylitis – 7%

Psoriasis – 9% (The prevalence of Axial disease in Psoriatic Arthritis patients varies between 25%-75% depending on the definition used which I suspect artificially lowers this number)

Iritis/Uveitis – 20-26%

Crohns/Ulcerative Colitis (IBD) – 4-10%

A number of studies have shown that investigating patients with Psoriasis/Uveitis/IBD and chronic back pain could identify a significant number of undiagnosed Spondyloarthropathies and reduce delay to diagnosis by up to 7 years.

As usual thanks for reading and I hope that you find this useful.

Please get back to me with any feedback, there will be more mini blogs like this one to interlude the longer ones.

Hypermobility Part 2 (syndrome?)

Intro

Hypermobility is common in Physio clinics the world over, over a series of blogs I hope to convey my current understanding of the factors at play with regards to diagnosis and management of these individuals. I would like to take this opportunity to say this is MY synthesis of available evidence and experience of working with these individuals. I am perfectly happy to be challenged and corrected as I feel I have a lot to learn about this highly complex group!

This blog will outline when we might consider the attending individual to have systemic effects related to their hypermobility warranting referral for medical review. If you didn’t read part 1 yet, please give that a look over first.

PLEASE REMEMBER – THIS BLOG IS NOT A REPLACEMENT FOR CLINICAL REASONING, IF YOU ARE UNSURE GET ADVICE

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For a number of years there have been guides on which symptoms to look out for in individuals who may have hypermobile joints that can help us as clinicians, the “Beighton” and “Brighton” scores are the most famed of these. The Beighton score looks at 9 areas for hypermobility and the higher the score the more likely a “generalised hypermobility” would be present. The Brighton score includes the Beighton but with the addition of extra-articular features such as skin  thus giving a higher likelihood of a “Hypermobility Syndrome”.

It is important to have an understanding of these associated symptoms and signs for a number of reasons. In people presenting without a diagnosis, recognition is key, it will help to ensure appropriate management. In those presenting with a pre-existing diagnosis it will help to understand the effects/side effects of proposed therapy interventions.

Aside from the obvious joint related symptoms such as pain and laxity, there are a LOT of potentially associated symptoms. Some of these will affect therapy interventions to a greater or lesser degree but we can explore that a later date. Lets concentrate on recognition and referral for now.

The hypermobility syndromes are a spectrum, it is certainly not necessary to refer every person with excess mobility or laxity in joints for a Rheumatologist review but it is important that we understand the complex blend of relating symptoms to the syndrome or them being a separate medical issue requiring investigation. As such my general rule is that symptoms suggestive of an autonomic issue or related to the gut then I deem these outside of my “wheelhouse” and refer out to appropriate medical professionals (usually a Rheumatologist).

In the era of the internet some will attend with a preconceived idea of a referral being necessary and in some cases this will be the correct course of action and in others it will be an over medicalisation of their condition but at this time the best course of action is to take this on a case by case basis.

These symptoms indicating an outward referral can mostly be found in the Brighton criterias “Minor Criteria” notably these include, multi joint dislocations or multiple dislocations of the same joint, Marfanoid habitus and skin related issues (stretchy, easy bruising, abnormal scarring). Not included in the score are autonomic issues namely POTS (postural orthostatic tachycardia syndrome) and intestinal dysmotility.

As such my advice really is the following: an onward referral is to be considered when an individual presents with multiple hypermobile joints (beighton 4+) and at least one of the symptoms listed above. The location of the referral will depend mostly on your available referral pathways so become familiar with these.

The third blog in this series will look at types of Hypermobility syndrome, I hope that this will help to combine this and part 1 to help with the clinical reasoning process. Please feel free to ask questions or point me in the direction of further information!

As usual thanks for reading and I hope that you find this useful.

Great resources are the following paper and the http://hypermobility.org website.

Castori, M., Tinkle, B., Levy, H., Grahame, R., Malfait, F. and Hakim, A., 2017, March. A framework for the classification of joint hypermobility and related conditions. In American Journal of Medical Genetics Part C: Seminars in Medical Genetics(Vol. 175, No. 1, pp. 148-157).

Hypermobility Part 1

Intro

Hypermobility is common in Physio clinics the world over, over a series of blogs I hope to convey my current understanding of the factors at play with regards to diagnosis and management of these individuals. I would like to take this opportunity to say this is MY synthesis of available evidence and experience of working with these individuals. I am perfectly happy to be challenged and corrected as I feel I have a lot to learn about this highly complex group!

This blog will outline when we observe joints which have excess mobility.

PLEASE REMEMBER – THIS BLOG IS NOT A REPLACEMENT FOR CLINICAL REASONING, IF YOU ARE UNSURE GET ADVICE

Hypermobility

Hypermobility is an observation not a diagnosis, it is also not synonymous with “instability” and it is important that as physios we understand this so we are able to effectively communicate this difference to patients.

The definition of hypermobility is (note remember “normal” has a huge variance):

“Joint hypermobility (JH) is the term universally accepted to define the capability that a joint (or a group of joints) has to move, passively and/or actively, beyond normal limits along physiological axes”1

Hypermobility as an observation can be classified in 2 main ways, distribution and pathophysiology.

The easiest to define is distribution. Usually when hypermobility is observed at a single or only a few joints then it is “localised hypermobility” this includes conditions such as Genu Recurvatum and may be bilateral. When there is hypermobility observable in multiple locations (and in theory) all 4 limbs and the axial skeleton the term “generalised hypermobility” is more applicable. It is possible to have hypermobility in all 4 limbs if the hands and feet are the only areas affected “peripheral joint hypermobility” is a term that could be used in this circumstance.

The pathophysiology of an individual’s hypermobility requires a detailed history. It can of course indicate an underlying systemic pathology but it can also be acquired following, surgery, injury (trauma), joint disease or training.

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Instability

Joint instability is the increased risk of dislocation or subluxation, while this can of course be caused by hypermobility the two can exist in isolation. Other causes of joint instability include trauma, acquired or congenital joint abnormality, muscle or neurological disorders.

Remember, these are observations and/or descriptors, they are NOT a diagnosis.

Practice as a physio for any period of time and you will see circumstances of asymptomatic hypermobility and instability. These findings alone are not sufficient to cause problems (usually pain) and in some circumstances are advantageous. Just go watch the gymnasts in the Olympics…!

It quickly becomes clear that an individual presenting with joint pains or other symptoms alongside hypermobility of joints is a complex picture prior even to consideration of there being a systemic issue at play. It is also easy to see how confusing it can become for not only the individuals affected but the clinicians aiming to help them manage their symptoms. There will be overlap with many presenting to healthcare with trauma on top of pre-existing hypermobility or all manner of other presentations.

I believe we do a disservice to individuals to not consider all these factors sufficiently prior to “diagnosing” or attributing specific complaints to these observations and I suspect in most cases a multidisciplinary approach would be necessary to fully grasp the implications for the individual.

Subsequent blogs in this series will look at the systemic features of “hypermobility syndromes”, different syndromes that may present and management. Please feel free to ask questions or point me in the direction of further information!

As usual thanks for reading and I hope that you find this useful.

Great resources are the following paper and the http://hypermobility.org website.

Castori, M., Tinkle, B., Levy, H., Grahame, R., Malfait, F. and Hakim, A., 2017, March. A framework for the classification of joint hypermobility and related conditions. In American Journal of Medical Genetics Part C: Seminars in Medical Genetics(Vol. 175, No. 1, pp. 148-157).

Polymyalgia Rheumatica

Intro

I have had a bit a glut of requests about Polymyalgia Rheumatica (PMR) recently so this blog aims to give an overview of the condition and non-pharmacological management for Physios.

PLEASE REMEMBER – THIS BLOG IS NOT A REPLACEMENT FOR CLINICAL REASONING, IF YOU ARE UNSURE GET ADVICE

Overview

PMR is an inflammatory condition, main symptoms are shoulder and/or pelvic girdle pain and stiffness. The mainstay of treatment is pharmacological usually involving steroids. There has been almost no research into the effectiveness of non-pharmacological interventions2.

Onset is very unlikely prior to the age of 50, peak onset is 65 with women being affected at 3x the likelihood of men. There shouldn’t be any muscle weakness at presentation and likely proximal muscle tenderness to palpation.

Clinical Presentation

Proximal bilateral muscle aching and/or morning stiffness lasting greater than 45 minutes (shoulders 90%) are the most prevalent symptoms. Associated symptoms include general malaise, depression and loss of appetite. A sibling with PMR significantly increases the risk of development of this condition.1

Blood tests should show a raised ESR of >40 and/or a raised CRP. Other bloods will help to exclude differential diagnoses due to the lack of specificity of symptom presentation (more bloods info here).1

There is no role for imaging in suspected PMR.

If PMR is suspected, refer to GP for appropriate management and/or onward referral to Rheumatology.

Physiotherapy Management

As mentioned in the intro there has been no research into the non-pharmacological management of PMR so I have no “evidence base” to present to you for management. The following is based on my clinical experience and adapted from evidence in other conditions.

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Physiotherapy management should focus on function restoration and general health. Obvious targets include, increased capacity for tasks involving the proximal muscles, cardiovascular health and general health education.

Function Restoration/Maintenance

It sounds obvious to say but this is really going to depend on the function required and the current deficits. Pacing advice is an option if there are no overt physical deficits such as loss of strength or range of motion.

Progressive loading programs will be useful to restore or maintain muscle strength, there is evidence confirming these are safe and effective in many other Inflammatory Conditions so there is nothing to lead me to believe PMR will be different. Be mindful of patients on titrating doses of steroids, I have seen individuals flare up as their dose reduces, ensure they have appropriate options with their exercise programs in case of this outcome.

General exercise programs are important in all inflammatory conditions. All the systemic inflammatory arthropathies (spondyloarthritis, Rheumatoid Arthritis and Psoriatic Arthritis) are associated with an increase in cardiovascular disease risk. The associated benefits of this type of program will help to mitigate some of this risk. Again, the evidence in other inflammatory conditions has shown even high intensity exercise programs to be safe. I advocate these programs to be patient preference led.

Education

Education is massive part of what we do as Physios and for PMR patients this is no different. Knowledge is power as they say and this is part of being able to self-manage any condition.

Condition Education is rather difficult in a condition with such a lack of information regarding pathophysiology but explaining how the inflammatory system is linked to immunity, repair and normal reactions to exercise can help patients to understand why they get the muscle soreness and stiffness associated with PMR. Hopefully this will lead to an understanding that exercise and activities, while not symptomless, are also not harmful to them in the long term. It can also aid in understanding when they have overdone it.

General Health Education includes smoking cessation, dietary advice and of course sleep. All three of these components are associated with outcomes in ALL long-term conditions as well as specifically inflammatory ones.

Closing

Hopefully this blog has given you some insight into recognising PMR if it presents in clinic (think Female, 50+, bilateral stiffness/pain, raised inflammatory markers) and some ideas on how to manage those already diagnosed.

References

  1. Book by Drs Al-Sukaini, Azam and Samanta https://www.amazon.co.uk/Rheumatology-clinical-handbook-medical-students/dp/1907904263/ref=sr_1_1?ie=UTF8&qid=1538037053&sr=8-1&keywords=rheumatology+a+clinical+handbook
  2. Dejaco C, Singh YP, Perel P, et al 2015 Recommendations for the management of polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative Annals of the Rheumatic Diseases 2015;74:1799-1807.

Inflammatory Arthropathy Bloods

Blood tests are a vital component when it comes to clinical reasoning in Rheumatology. Not everyone has the authorisation to order bloods for their patients but a working knowledge remains important to help you in management of a suspected rheumatology patient. This blog will outline the basic bloods for the most common arthropathies (i.e. it leaves out connective tissue disorders and myopathies etc.) It is important to remember here that interpretation is nuanced, requires clinical correlation and that formal diagnosis remains the role of Rheumatologists with this group of patients. Conditions will always remain “suspected” until investigation by a Rheumatologist.

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PLEASE REMEMBER – THIS LIST IS NOT A REPLACEMENT FOR CLINICAL REASONING, IF YOU ARE UNSURE GET ADVICE

Routine blood tests will always be carried out for suspected Inflammatory Arthropathies such as Rheumatoid Arthritis and the Spondyloarthropathies. These will consist of a Full Blood Count, inflammatory markers, biochemical tests and immunological tests. This blog will concentrate on the inflammatory markers and biochemical tests. It is by no means exhaustive and is designed to help Physiotherapists to order appropriate blood tests. Again I reiterate, if you are unsure what to order, please seek advice.

Inflammatory Markers

These will often be raised in Inflammatory Arthropathies but remember many, many other things can cause increased levels of inflammatory markers in the blood (e.g. obesity, acute injury, infection) and  a negative result does not rule out a suspected condition on its own.

ESR (Erythrocyte Sedimentation Rate) – non specific test of inflammation

Normal range – Men 0-14, Women 0-20. Note that there is some variance with age so proceed with caution when interpreting if the levels are near to the top of these ranges.

CRP (C-reactive protein) – non specific test of inflammation

Normal range – <5. Note that this is very reactive to any activation of the immune system.

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Immunological Tests

Immunological tests have much more utility in diagnosis and can have value with prognosis in inflammatory arthropathies. Just a reminder that these still have diagnostic limitations. I would recommend this book by Drs Al-Sukaini, Azam and Samanta (https://www.amazon.co.uk/Rheumatology-clinical-handbook-medical-students/dp/1907904263/ref=sr_1_1?ie=UTF8&qid=1538037053&sr=8-1&keywords=rheumatology+a+clinical+handbook) as it has a brilliant breakdown of the sensitivity and specificity of these tests for the various diagnoses.

Rheumatoid Factor (RF) – note 15-20% of RF+ve patients have no associated conditions

Rheumatoid Arthritis – Sensitivity 60-90%, specificity 70-80%, higher values prognostic of poorer outcome.

Psoriatic Arthritis – A negative RF result has diagnostic utility in PsA

Anti-cyclic citrullinated peptide (Anti-CCP)

Rheumatoid Arthritis – highly specific 95%+ for diagnosing RA

HLA-B27 – Note present in roughly 8% of the population

Spondyloarthropathy – 90% of diagnosed SpA patients will be positive for the HLA-B27 gene

Psoriatic Arthritis – 40%+ will be positive for the HLA-B27 gene

I hope that this gives you a good reference point to aid with clinical reasoning. Remember that due to a lack of sensitivity and specificity these blood tests are best used in conjunction with clinical correlation. It would be a rare case indeed to refer (or not refer) to Rheumatology purely based upon a positive/negative blood result. Use your clinical judgement and seek advice where needed.

Also remember this list does not cover many other types of Rheumatology conditions!

Other resources to help you include the mentioned clinical handbook and my “suspecting RA tool” which is free to download here