STAGES OF CRPS/RSD
CRPS/RSD has been divided into
different stages. Depending on nature of injury, the stages vary in their
duration. In the 17 patients suffering from venipuncture CRPS in our series,
deterioration from stage I to stage III was measured in a few weeks up to less
than 9 months. This is in contrast with CRPS in children in whom stages would
stagnate, reverse or improve slowly.
In STAGE I , is a
sympathetic dysfunction with typical thermatomal distribution of the pain.
The pain may spread in a mirror fashion to contralateral extremity or to
adjacent regions on the same side of the body. In stage one, the pain is
usually SMP in nature.
In STAGE II, the
dysfunction changes to dystrophy manifested by edema, hyperhidrosis,
neurovascular instability with fluctuation of livedo reticularis and
cyanosis - causing change of temperature and color of the skin in matter of
minutes. The dystrophic changes also include bouts of hair loss, ridging,
dystrophic, brittle and discolored nails, skin rash, subcutaneous bleeding,
neurodermatitis, and ulcerative lesions. Due to the confusing clinical
manifestations, the patient may be accused of factitious self-mutilation and
"Münchausen syndrome." All these dystrophic changes may not be present at
the same time nor in the same patient. Careful history taking is important
in this regard.
In STAGE III, the
pain is usually no longer SMP and is more likely a sympathetically
independent pain (SIP). Atrophy in different
degrees is seen. Frequently, the atrophy is overshadowed by subcutaneous
edema. The complex regional pain and inflammation spread to other
extremities in approximately 1/3 of CRPS patients. At stage II or III it is
not at all uncommon for CRPS to spread to other extremities. At times, it
may become generalized. The generalized CRPS is an infrequent late stage
complication. It is accompanied by sympathetic dysfunction in all four
extremities as well as attacks of headache, vertigo, poor memory, and poor
concentration. The spread through paravertebral and midline sympathetic
nerves may be vertical, horizontal, or both. The original source of CRPS may
sensitize the patient to later develop CRPS in another remote part of the
body triggered by a trivial injury. The ubiquitous phenomenon of referred
pain to remote areas (e.g., from foot or hand to spine) should not be
mistaken for the spread of CRPS.
At STAGE III, inflammation becomes
more problematic and release of neuropeptides from c-fiber terminals results
in multiple inflammatory and immune dysfunctions. The secondary release of
substance P may damage mast cells and destroy muscle cells and fibroblasts.
1. Failure of the immune system,
reduction of helper T-cell lymphocytes and elevation of killer T-cell
2. Intractable hypertension changes to orthostatic
3. Intractable generalized edema involving the
abdomen, pelvis, lungs, and extremities.
4. Ulcerative skin lesions which may
respond to treatment with I.V. Mannitol, I.V. Immunoglobulin, and ACTH
treatments. Calcium channel blockers such as
Nifedipine may be effective in treatment.
5. High risks of cancer and suicide are increased.
6. Multiple surgical procedures seem to be
precipitating factors for development of stage IV.
Stage IV is
almost the flip side of earlier stages, and points to exhaustion of
autonomic and immune systems. Ganglion blocks in this stage are useless and
treatment should be aimed at improving the edema and the failing immune
system. Sympathetic ganglion blocks, alpha blockers, including Clonidine,
are contraindicated in stage IV due to hypotension. Instead, medications
such as Proamantin (midodrin) are helpful to correct the orthostatic