Does a familial subtype of complex regional pain syndrome exist? Results of a systematic review

Figures & data

Figure 1. Flow diagram of the study selection process.

Table 1. Summary of study details for papers included in this systematic review (n = 8).

Study, country	Sample size	Mean age (SD)	% Female	Region of pain	Known cause?	CRPS diagnostic criteria	% fCRPS	Method of study	Type of family relationship	Quality (%)
Albert and Ott,^Citation26 Switzerland	3 fCRPS	37 (5.9)	0	Hips	No	n/r	100	Case report	Sibling	87
de Rooji et al,^Citation31 The Netherlands	405 CRPS (38 fCRPS)	40.6 (15.3)	85	52% arm, 48% leg	13% no cause, 87% trauma, 22% fracture, 27% surgery, 13% soft tissue, 25% other	IASP	9.4	Case–control	Sibling	62
de Rooji et al,^Citation33 The Netherlands	84 fCRPS	36.7 (14.5)	83	49% arm, 44% leg, 7% both	23% no cause, 77% trauma, 27% fracture, 20% surgery, 20% soft tissue, 9% other	IASP	100	Case–control	Any blood relative	54
Veldman et al.,^Citation29 The Netherlands	829 CRPS (5 fCRPS)	Varied between 9 and 85; median: 42	CRPS: 76	CRPS: 59% arm, 41% leg	10% no cause, 65% trauma (mostly fracture), 19% surgery, 15% inflammatory process, 4% other (injection, intravenous infusion, cerebrovascular accident)	Specific diagnostic criteria (similar to IASP)	0.6	Case series	Any blood relative	90
Erdmann and Wynn-Jones,^Citation27 England	2 fCRPS	35.5	50	Both cases: hands	1: injury–slipped 1: injury–vehicle accident	n/r	100	Case report	Sibling	87
Shirani et al,^Citation28 United States	69 CRPS (9 fCRPS)	fCRPS: 33.8 (12)	fCRPS: 70	77.8% arm, 22.2% leg	22% no cause, 78% trauma or surgery	IASP	13	Case series	Immediate family (parents/siblings)	90
Higashimoto et al.,^Citation30 United States	8 CRPS	1 at birth; the rest: 6.3 (4.8)	n/r	37.5% leg, 12.5% arm, 37.5% both, 12.5% n/r	28% no cause 2: surgery 1: traumatic fracture 1: a fall	IASP	25; the rest unclear	Case series	2 siblings	90
Huhne et al.,^Citation32 Germany	60 (12 fCRPS); 21 unaffected relatives	n/r	fCRPS: 92, sCRPS: 71	n/r	100% known cause—traumatic fracture or surgery	IASP	20	Case–control	Immediate family	76

CRPS = complex regional pain syndrome; fCRPS = familial complex regional pain syndrome; n/r = not reported; IASP = International Association for the Study of Pain; sCRPS = sporadic complex regional pain syndrome.

Albert J, Ott H. Three brothers with algodystrophy of the hip. Ann Rheum Dis. 1983;42(4):421–24. doi:10.1136/ard.42.4.421.

 PubMed Web of Science ®Google Scholar

de Rooij AM, de Mos M, van Hilten JJ, Sturkenboom MCJM, Gosso MF, van Den Maagdenberg AMJM, Marinus J. Increased risk of complex regional pain syndrome in siblings of patients? J Pain. 2009;10(12):1250–55. doi:10.1016/j.jpain.2009.05.006.

 PubMed Web of Science ®Google Scholar

de Rooij AM, de Mos M, Sturkenboom MCJM, Marinus J, van den Maagdenberg AMJM, van Hilten JJ. Familial occurrence of complex regional pain syndrome. Eur J Pain. 2009;13(2):171–77. doi:10.1016/j.ejpain.2008.04.004.

 PubMed Web of Science ®Google Scholar

Veldman PH, Reynen HM, Arntz IE, Goris RJ. Signs and symptoms of reflex sympathetic dystrophy: prospective study of 829 patients. Lancet (London, England). 1993;342(8878):1012–16. doi:10.1016/0140-6736(93)92877-v.

 PubMed Web of Science ®Google Scholar

Erdmann MW, Wynn-Jones CH. “Familial” reflex sympathetic dystrophy syndrome and amputation. Injury. 1992;23:136–38.

 PubMed Web of Science ®Google Scholar

Shirani P, Jawaid A, Moretti P, Lahijani E, Salamone AR, Schulz PE, Edmondson EA. Familial occurrence of complex regional pain syndrome. Can J Neurol Sci. 2010;37:389–94.

 PubMed Web of Science ®Google Scholar

Higashimoto T, Baldwin EE, Gold JI, Boles RG. Reflex sympathetic dystrophy: complex regional pain syndrome type I in children with mitochondrial disease and maternal inheritance. Arch Dis Child. 2008;93(5):390–97. doi:10.1136/adc.2007.123661.

 PubMed Web of Science ®Google Scholar

Huhne K, Leis S, Schmelz M, Rautenstrauss B, Birklein F. A polymorphic locus in the intron 16 of the human angiotensin-converting enzyme (ACE) gene is not correlated with complex regional pain syndrome I (CRPS I). Eur J Pain. 2004;8(3):221–25. doi:10.1016/j.ejpain.2003.08.004.

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