CAPILLAROSCOPIC FINDINGS IN ANTIPHOSPHOLIPID SYNDROME
Authors: Doncheva – Dilova Jaklin , Boyadzhieva Vladimira , Emin Soner , Stoilov Nikolay 
Published: December 2024

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial and/or venous thrombosis and high levels of antiphospholipid antibodies (aPL). The disease mainly affects women over 30 years of age and can occur as a primary syndrome (PAPS) or in the context of another autoimmune disease (secondary APS). Antiphospholipid antibodies (aPL) can cause blood clots to form in the veins and arteries and lead to heart attack, stroke, pulmonary embolism, preeclampsia, miscarriage, slow growth or damage to the fetus, premature / stillbirth. Risk factors include arterial hypertension, obesity, hypercholesterolemia and hypertriglyceridemia, smoking, atherosclerosis, taking birth control pills or associated autoimmune diseases (such as systemic lupus, SLE). In patients with PAPS, capillary hemorrhages are associated with neurological manifestations and with comorbidity (most often with hypertension). In some people, the syndrome is asymptomatic, while in others the disease may present with deep vein thrombosis, bleeding from the nose and gums, unusually heavy menstruation, hematemesis, melena. Complications include pulmonary embolism, stroke, heart attack or transient ischemic attack in young people without risk factors for cardiovascular disease, kidney failure, miscarriages, etc. Treatment aims to reduce the risk of blood clots through anticoagulant therapy. Raynaud's phenomenon (RP) represents the most common clinical aspect of microvascular involvement and is a key feature of diseases such as systemic sclerosis (SSc), mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), dermatomyositis (DM), polymyositis (PM) and antiphospholipid syndrome (APS). Nail bed capillaroscopy (NVC) is an extremely useful, noninvasive, and reliable method for analyzing microvascular changes in Raynaud's phenomenon and systemic diseases, as well as for determining disease severity and prognosis. The presence of autoantibodies or capillaroscopic changes are essential for the early diagnosis of APS. It is not well established whether patients with primary antiphospholipid syndrome (PAPS) have specific capillaroscopic findings, but patients with positive IgG and IgM anticardiolipin antibodies have a higher incidence of capillaroscopic changes compared to healthy controls. Changes in capillary length and the presence of symmetrical microhemorrhages have been described, which are associated with thrombotic events, suggesting direct damage to the vascular endothelium caused by antiphospholipid antibodies. Other changes described include slight disorganization of capillary arrangement, change in capillary morphology (dilated, branched, kinked, bushy), change in capillary loop length and shape (apical coiling, single crossing between loops with moderate coiling and double crossing between loops ), avascular zones and neoangiogenesis. Many authors emphasize the presence of an "atypical normal" capillaroscopic pattern or "non-specific capillaroscopic changes". Other authors, however, did not reveal a similar capillaroscopic pattern in patients with anticardiolipin antibodies. Oral anticoagulants can be the cause of a high frequency of isolated microhemorrhages, which is why it is mandatory to pay special attention to patients treated with them, with a view to specifying the reasons for the finding. Nevertheless, capillaroscopy remains a valuable diagnostic tool in the differential diagnosis of many connective tissue diseases (occurring with or without Raynaud's phenomenon) as well as in aiding the diagnosis of antiphospholipid syndrome (APS), as cases of seronegative (regarding antiphospholipid antibodies) patients with clinical manifestations of APS.