Types of CRPS

There is now one main type of Complex Regional Pain Syndrome (CRPS) and three subtypes according to The Valencia consensus-based adaptation of the IASP Complex Regional Pain Syndrome Diagnostic Criteria (2021). These are:

Main Type:

• Type 1 or CRPS I

Subtypes:

• Type 2 or CRPS II
• CRPS - Not Otherwise Specified (CRPS-NOS)
• CRPS with Remission of Some Features

Even though there are the two main types of CRPS, the signs and symptoms are almost the same for both.

The terms Warm/Hot CRPS, Intermediate CRPS and Cold/Blue CRPS are not classed as Complex Regional Pain Syndrome sub-types, but more of an explanation of the stages that a person living with CRPS goes through in each type, i.e. from acute phase to chronic phase.

Type 1 or CRPS I

Type 1 or CRPS I used to be known as Reflex Sympathetic Dystrophy (RSD). There are still many people including doctors and specialists who still refer to CRPS as RSD. Type 1 or CRPS I occurs after an illness or an injury that did NOT directly damage a nerve. It usually occurs after a minor or major tissue injury to the extremities. Type 1 will usually develop from injuries including (but not limited to) soft tissue damage, sprains or fractures (not nerve related).

According to the Mayo Clinic’s web page for Complex Regional Pain Syndrome, "About 90 percent of people with complex regional pain syndrome have type 1."

Type 2 or CRPS II

Type 2 or CRPS II used to be known as Causalgia and occurs after there has been an actual known injury to a nerve.

CRPS-NOS

According to the Budapest Criteria, there is another diagnostic type of CRPS, or rather a sub-type of CRPS. This is known as CRPS – Not Otherwise Specified or CRPS-NOS. This third sub-type of CRPS, CRPS-NOS, was added because approximately 15% of patients previously diagnosed with CRPS (based on 1994 IASP criteria) would not fully meet the new clinical diagnostic Budapest Criteria (Sebastin, S.J. et al 2011).

According to the RCP UK guidelines (2018), CRPS-NOS is a type of CRPS: "…for patients who do not fully meet the criteria but whose signs and symptoms could not be explained better by another diagnosis. For patients who fulfilled the Budapest criteria in the past but no longer do so, the term CRPS-NOS may also be used."

CRPS with Remission of Some Features

This is a new sub-type as agreed under the Valencia Consensus, which overlaps with either CRPS I or Il.

This sub-type is for those patients who have been previously diagnosed or documented as having fully met the CRPS criteria (either CRPS I or CRPS II) but who are currently not displaying sufficient CRPS symptoms to fully meet the diagnostic criteria

Warm/Hot, Intermediate or Cold/Blue Complex Regional Pain Syndrome (CRPS)

You may also hear other expressions concerning Complex Regional Pain Syndrome (CRPS), such as: Warm / Hot CRPS, Intermediate CRPS and Cold/Blue CRPS. These three terms are seen as diagnostic terms or sub-groups of CRPS, rather than symptoms of the condition.

Warm/Hot/Red CRPS

The Warm or Hot type of CRPS is seen by doctors and specialists as the 'acute' phase of the affected CRPS limb or CRPS area. The majority of CRPS sufferers will have this Warm or Hot type, with 70% of CRPS classed as having the Warm or Hot type CRPS compared to 30% with the Cold CRPS (Eberle, T et al 2009).

According to a recent study by Dirckx, M et al (2015), for the warmer CRPS side: "…inflammation is generally assumed to be present."

If diagnosis is made early or if symptoms have appeared soon after injuries, surgery or otherwise, then the term of hot or cold is sometimes used by doctors and specialists. There will usually be a distinct temperature difference between the CRPS affected limb and the non affected limb or limbs. Most crps sufferers won’t have been told of their hot or cold type, possibly because diagnosis was too late.

At the initial stages of Complex Regional Pain Syndrome, sufferers with CRPS Type I will often have a warmer affected limb. However, eventually when the Type I becomes chronic, the CRPS affected limb will then turn cold. In the Warm/Hot phase of CRPS, it will often display the classic signs of inflammation (Veldmen, P.H. et al 1993). You will also find a greater pressure hyperalgesia on the affected CRPS limb or area (Vaneker, M. et al 2005).

According to Wasner, G et al. (2001): "…in CRPS I, unilateral inhibition of sympathetic vasoconstrictor neurones leads to a warmer affected limb in the acute stage."

Intermediate CRPS

Intermediate Complex Regional Pain Syndrome (CRPS) is where the CRPS affected limb or area is neither Warm/Hot nor Cold/Blue (Wasner, G. et al 2001) (Dirckx, M et al 2015).

Cold or Blue CRPS

The term Cold CRPS or Blue CRPS is usually is referred to in the chronic stages of Complex Regional Pain Syndrome. Bruehl, S. et al (2010) found that the transition from Warm/Red CRPS to Cold/Blue is common in CRPS. CRPS patients with this type will have lower McGill Pain Questionnaire (MPQ) results, showing that there is increased central nervous system involvement as well as a higher chance of dystonia.

If you have been diagnosed with CRPS Type II (also known as Causalgia) you will most likely have had a diagnosis much earlier than patients with CRPS Type I. This is because for those living with CRPS Type II, there has been a known direct injury to a nerve, therefore it will increase the probability of vasoconstriction causing the cold temperature straight away.

Unfortunately, according to a study by Vaneker, M. et al (2005), "…cold CRPS 1 patients have poorer clinical pain outcomes and show persistent signs of central sensitisation correlating with disease progression. The latter is not the case for warm CRPS 1 patients."

In the research study by Wasner, G. et al (2001), they said oft the cold CRPS Type I: "Secondary changes in neurovascular transmission may lead to vasoconstriction and cold skin in chronic CRPS I, whereas sympathetic activity is still depressed."

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