Acrosyndromes (Raynaud's phenomenon and related disorders)

• In this situation, no laboratory test is recommended

Laboratory tests

• Antinuclear antibodies (ANA) (to rule out early connective tissue disease)if neededThe ESVM (Europe, 2017) requests them in every patient with Raynaud's phenomenon ^[2]; in France, the rare diseases network, the SFMV and the COFER do too, together with capillaroscopy, whereas the Collège de médecine interne does not recommend any tests for a typical presentation ^[3]. Their absence at baseline is the factor most associated with non-progression to scleroderma at 5 years (European VEDOSS cohort) ^[4]. A low titre (1/40, for example) does not rule out a primary form ^[5].
• FBCif neededThe ESVM requests them in every patient with Raynaud's phenomenon, ESR being able to replace CRP (expert opinion) ^[2]; in France, the rare diseases network, the SFMV and the COFER list them as “±” ^[3]. A normal CRP does not rule out a connective tissue disease ^[2].
• CRPif neededThe ESVM requests them in every patient with Raynaud's phenomenon, ESR being able to replace CRP (expert opinion) ^[2]; in France, the rare diseases network, the SFMV and the COFER list them as “±” ^[3]. A normal CRP does not rule out a connective tissue disease ^[2].

Laboratory tests

• FBC
• CRP
• Antinuclear antibodies (ANA)
• TSHif neededDepending on the clinical context: the rare diseases network, the SFMV and the COFER list them as “±” without specifying the situation ^[3].
• rheumatoid factorif neededDepending on the clinical context: the rare diseases network, the SFMV and the COFER list them as “±” without specifying the situation ^[3].

Urine tests

• Urine dipstick (to look for renal involvement)if needednon-French guidelineThe ESVM (Europe, 2017) considers it useful to detect renal involvement, for example in lupus ^[2]; a US-UK review lists it among the tests often requested ^[4]. The tests of the rare diseases network, the SFMV and the COFER do not mention it ^[3].

Laboratory tests

• Anti-Scl70
• Anti-centromere
• Anti-RNP
• Anticardiolipin
• Cryoglobulins

Laboratory tests

• FBC
• Antinuclear antibodies (ANA) (if signs of rheumatic or autoimmune disease)if neededClinical signs suggesting a rheumatic or autoimmune disease: secondary erythromelalgia can accompany these diseases ^[10]. Together with the FBC and the search for thyroid dysfunction, antinuclear antibody testing helps distinguish a primary form from a secondary one; erythromelalgia can accompany lupus ^[8].
• TSH (if signs of hyperthyroidism)if neededClinical signs of hyperthyroidism: erythromelalgia can, more rarely, accompany hyperthyroidism, and the search for thyroid dysfunction is among the tests that distinguish primary from secondary forms ^[8].

If the FBC is normal: to be repeated regularly

• FBC