DOI: 10.1515/folmed-2016-0011 REVIEW The Place of Nailfold Capillaroscopy Among Instrumental Methods for Assessment of Some Peripheral Ischaemic Syndromes in Rheumatology Sevdalina N. Lambova Department of Propedeutics of Internal Medicine, Faculty of Medicine, Medical University of Plovdiv, Plovdiv, Bulgaria Correspondence: Sevdalina Lam- Micro- and macrovascular pathology is a frequent fi nding in a number of common bova, Department of Propedeutics rheumatic diseases. Secondary Raynaud’s phenomenon (RP) is among the most in Internal Medicine, Faculty of common symptoms in systemic sclerosis and several other systemic autoimmune Medicine, Medical University of diseases including a broad diff erential diagnosis. It should be also diff erentiated Plovdiv, Plovdiv, 15A Vassil Aprilov from other peripheral vascular syndromes such as embolism, thrombosis, etc., Blvd., 4002 Plovdiv, Bulgaria E-mail: [email protected] some of which lead to clinical manifestation of the blue toe syndrome. Tel: +359 889 560 104 The current review discusses the instrumental methods for vascular assessments. Received: 9 March 2016 Nailfold capillaroscopy is the only method among the imaging techniques that Accepted: 4 May 2016 can be used for morphological assessment of the nutritive capillaries in the nail- Published: 30 June 2016 fold area. Laser-Doppler fl owmetry and laser-Doppler imaging are methods for Key words: Raynaud’s phenome- functional assessment of microcirculation, while thermography and plethysmog- non, blue toe syndrome, rheu- raphy refl ect both blood fl ow in peripheral arteries and microcirculation. Doppler matic diseases, microcirculation; ultrasound and angiography visualize peripheral arteries. The choice of the appro- nailfold capillaroscopy priate instrumental method is guided by the clinical presentation. The main role of Citation: Lambova SN. The capillaroscopy is to provide diff erential diagnosis between primary and secondary Place of Nailfold Capillaroscopy RP. In rheumatology, capillaroscopic changes in systemic sclerosis have been re- Among Instrumental Methods for cently defi ned as diagnostic. The appearance of abnormal capillaroscopic pattern Assessment of Some Peripheral inherits high positive predictive value for the development of a connective tis- Ischaemic Syndromes in Rheuma- sue disease that is higher than the predictive value of antinuclear antibodies. In tology. cases of abrupt onset of peripheral ischaemia, clinical signs of critical ischaemia, Folia Medica 2016;58(2);77-88, unilateral or lower limb involvement, Doppler ultrasound and angiography are doi: 10.1515/folmed-2016-0011 indicated. The most common causes for such clinical picture that may be referred to rheumatologic consultation are the antiphospholipid syndrome, mimickers of vasculitides such as atherosclerosis with cholesterol emboli, and neoplasms. INTRODUCTION RESULTS Micro- and macrovascular pathology is a common RAYNAUD’S PHENOMENON feature in a number of common rheumatic diseases. Raynaud’s phenomenon (RP) is among the most Reliable diagnostic tools should be used in order to common symptom in systemic autoimmune diseases. guide physicians in the differential diagnosis and It manifests as reversible vasospasm of the small administration of well-timed treatment that would peripheral arteries and arterioles at cold exposure or improve prognosis, slow up or prevent future dam- emotional stress. It presents in three or two phases age or provide full recovery in cases of medical e. g., ischaemia, asphyxia and reactive hyperemia emergency. with skin discolouration from pallor to cyanosis and redness with clearly visible demarcation line. It AIM affects acral body parts such as fi ngers, toes, nose, The present review discusses the instrumental ears, lips. RP could be idiopathic or primary, when methods for vascular assessment. Two case reports there is no underlying cause for its development and are presented to illustrate the possibilities of the the clinical course is benign without development of imaging modalities in clinical context. trophic changes because of the lack of endothelial 77 Folia Medica I 2016 I Vol. 58 I No. 2 I Article 1 S. Lambova damage. Secondary RP is observed in a number of to the higher use of different vascular interventions connective tissue diseases and includes a broad dif- such as vascular surgery or invasive percutaneous ferential diagnosis including drug-induced RP and procedures i.e., angiography and angioplasty. In paraneoplastic conditions. The profound endothelial addition, anticoagulant therapy could also induce damage may lead to appearance of digital ulcers this iatrogenic complications.6 in a proportion of cases with secondary RP.1-4 It A number of instrumental techniques are used should be also differentiated from other peripheral to assess microcirculation in patients with symp- vascular syndromes such as embolism, thrombosis, toms of RP in order to prove the presence of etc., some of which lead to clinical manifestation vascular pathological changes and to differentiate of the blue toe syndrome. A number of laboratory the primary from secondary forms of the disease and instrumental methods are essential for the dif- that is crucial for the therapeutic approach such ferential diagnosis. Interpretation of the fi ndings is as nailfold capillaroscopy, laser Doppler fl owmetry, in the concrete clinical context. laser Doppler imaging, thermography at rest and in RP is diagnosed clinically. The diagnosis is combination with cold provocation. Colour Doppler based on the direct observation of 2 among the 3 and angiography are additional instrumental tech- possible phases of the condition. To be diagnosed niques that visualize larger vessels and facilitate with RP, a patient should have a history of sensi- the differential diagnosis in more complex cases tivity to the cold and episodic pallor, cyanosis or of peripheral vascular ischaemic pathology. redness of the distal portions of the digits after Nailfold capillaroscopy is the only method for exposure to cold. Photographs of the hands may morphological assessment of nutritive capillaries. be obtained during an attack and used to confi rm Laser Doppler and thermography assess cutaneous the history. In routine clinical practice it is not blood vessel function.7 necessary to perform a cold provocation test to RAYNAUD’S PHENOMENON IN RHEUMATIC DISEASES make a defi nitive diagnosis of RP.5 RP is a characteristic feature in a number of rheu- BLUE TOE SYNDROME matic diseases with substantially high frequency in The term ‘blue toe syndrome’ describes the devel- systemic sclerosis (SSc) - approximately 90-95% opment of blue or violaceous discoloration of one (Table 1).8,9 RP in SSc is a clinical manifesta- or more toes in the absence of obvious trauma, tion of vasospasm of the peripheral digital arteries serious cold-induced injury, or disorders producing and arterioles as well as of permanent structural generalized cyanosis. The condition may develop in endothelial damage of the microcirculation that is cases of micro-, macrovascular pathology, impaired associated with the high rate of development of venous outfl ow and hyperviscosity syndromes. Three digital ulcers in these patients in over half of the major categories are recognized: cases.10 In 100 SSc patients, La Montagna et al. 1. disorders with decreased arterial fl ow - arterial (2002) found that symptoms of RP of the feet obstruction due to: are present in 90% of the cases vs 100% fre- • embolism (atheroemboli due to spontaneous plaque quency of RP at the hands. In 43% of the cases haemorrhage or induced by different provoking from this group, RP of the feet was presented at factors, cardiac or aortic tumor, infective endo- initial evaluation, while 47% developed it in the carditis); course of the follow-up. In contrast, RP of the • thrombosis (antiphospholipid syndrome, malig- hands was registered in 100% of patients at the nancy (paraneoplastic acral vascular syndrome, initial evaluation. The onset of clinically evident disseminated intravascular coagulation, warfarin involvement of the feet was found to occur later skin necrosis); in limited SSc than in the diffuse form of the • vasoconstrictive disorders (acrocyanosis, chil- disease. Lower rate of necrotizing RP was found blains, drug induced vasoconstriction, infectious and non-infectious vessel infl ammation; in the feet of patients with SSc as compared with 11 2. impaired venous outfl ow (venous thrombosis); the hands. 3. abnormal circulating blood (paraproteinemia Among other common rheumatic diseases, the with hyperviscosity, myeloproliferative disorders, prevalence of RP in systemic lupus erythematosus cryofi brinogenemia, cryoglobulinemia). varies between 10 and 45% and it usually indicates 8,12 Prevalence of atheroembolism is increased due a more benign course without tissue necrosis. 78 Folia Medica I 2016 I Vol. 58 I No. 2 I Article 1 Instrumental Methods for Assessment of Peripheral Ischaemic Syndromes in Rheumatology Table 1. Common differential diagnosis of Raynaud’s phenomenon in rheumatologic practice. Systemic sclerosis Mixed connective tissue disease Undifferentiated connective tissue disease Systemic lupus erythematosus Dermatomyositis, polymyositis Sjögren syndrome Rheumatoid arthritis Systemic vasculitides - Buerger disease, Takayasu arteritis, polyarteritis nodosa, granulomatosis with poly- angiitis, etc. Other common conditions that are associated with Raynaud’s phenomenon and are included in the differential diagnosis in rheumatologic