Comment on: Cryofibrinogenaemia —a neglected disease

Sir, We read with great interest the review of Moiseevet al. [1] on cryofibrinogenaemia (CF). However, we have some concern over the clinical significance of this condition. First, we were intrigued by some discrepancies between the different prevalences of essential (or primary) and secondary, asymptomatic and symptomatic CF. Indeed, although symptomatic and essential CF is extremely rare, with only ∼210 cases published from 1964 to 2017 (data not shown), CF presence has been found in 3–7% of healthy controls [2,3] and in 3 –13% of hospitalized patients [3]. In these cases , CF has been described in association with a wide array of conditions. These correlated diseases are autoimmune (such as SLE and ANCA vasculitides), infectious (bacterial and viral), inflammatory (Crohn ’s disease), atherosclerotic, thromboembolic, drug-related (oral contraceptives), metabolic (diabetes mellitus, hypothyroidism), neoplastic (both solid and haematological) and miscellaneous (chronic lung disease, hypothermia) [1,2,4]. It is difficult to imagine a pathophysiological connection between CF and all these diseases, whereas incidental association could not be ruled out. Indeed, CF is classically associated with cutaneous ulcerated thrombi [1,5]. However, a study including 49 patients with CF found that thrombotic events were equally distributed between lower extremity gangrene, myocardial infarction and pulmonary embolism [6], making it difficult to determine whether CF is the cause or t...
Source: Rheumatology - Category: Rheumatology Source Type: research