原发性关节软骨钙质沉着症的分类。

Czech Med. 1979;2(3):89-103.

Based on long-term observations the authors submit a categorization of primary (hereditary and solitary) articular chondrocalcinosis into three different sub-populations. Attention is drawn to the fact that the extent of the qualitative disorder of the articular cartilage, obviously conditioned genetically, is linked with the age factor and determines the quantitative differences of pyrophosphate arthropathy in primary chondrocalcinosis. In young age, as a rule in the third decade, severe polyarticular condrocalcinosis (first sub-population) develops which causes relatively soon invalidity, in middle age (5th and 6th decade) milder condrocalcinosis develops (second sub-population) which combines with extraarticular, tendinous and tissue calcifacations, and finally in advanced age oligoarticular chondrocalcinosis develops (third sub-population) which is usually associated with ankylosing hyperostosis of the spine. Articular chondrocalcinosis (CCA) which we described by this term as a special metabolic arthropathy which occurs in families and solitary and which we defined as a special nosological unit (35, 36,) has become generally known and firmly established in rheumatology. As ensues from numerous publications, primary (idiopathic) CCA which comprises the hereditary and solitary (sporadic) form is characterized by pyrophosphate arthropathy which develops on articular cartilages not damaged by another process (13, 25, 26, 37); on the other hand as secondary CCA we consider pyrophosphate arthropathies which are associated with metabolic, endocrine or other diseases (9, 30). The common sign of both basic forms of CCA is the presence of microcrystals of calcium pyrophosphate dihydrate (CaPD) in articular cartilages, synovial fluid, or other articular structures (capsules, tendons, ligaments), characterized originally by McCarty et al. (11, 18) and later by other authors (2, 23, 27, 32). In addition to semantic (terminological) problems there were also questions of the classification of CCA because, based on an analysis of major groups of patients, it was revealed that there is a varied picture of chondrocalcinosis or pseudogout (18, 19) or deposition disease (17). of calcium pyrophosphate dihydrate crystals. In our paper we are presenting our view on the primary form of CCA and submitting the characteristics of classification which ensued from more than 20 years observation of our group of patients, in particular based on the evaluation of the beginning of the clinical and X-ray manifestations and the further development of the disease.

基于长期观察，作者将原发性（遗传性和散发性）关节软骨钙质沉着症分为三个不同的亚组。需要注意的是，关节软骨定性紊乱的程度显然由遗传因素决定，它与年龄因素相关，并决定了原发性软骨钙质沉着症中焦磷酸关节病的定量差异。在年轻时，通常是在第三个十年，会发生严重的多关节软骨钙质沉着症（第一亚组），这会相对较快地导致残疾；在中年（第五和第六个十年），会出现较轻的软骨钙质沉着症（第二亚组），它与关节外、肌腱和组织钙化同时出现；最后，在老年时会发生少关节软骨钙质沉着症（第三亚组），它通常与脊柱强直性骨质增生有关。我们将这种关节软骨钙质沉着症（CCA）描述为一种特殊的代谢性关节病，它可发生于家族性和散发性情况，并将其定义为一个特殊的病种单位（35, 36），这在风湿病学中已广为人知并牢固确立。从众多出版物中可以看出，原发性（特发性）CCA包括遗传性和散发性（偶发性）形式，其特征是焦磷酸关节病发生在未受其他病变损害的关节软骨上（13, 25, 26, 37）；另一方面，我们将与代谢、内分泌或其他疾病相关的焦磷酸关节病视为继发性CCA（9, 30）。CCA两种基本形式的共同特征是在关节软骨、滑液或其他关节结构（关节囊、肌腱、韧带）中存在二水焦磷酸钙（CaPD）微晶，最初由麦卡蒂等人（11, 18）描述，后来其他作者也有描述（2, 23, 27, 32）。除了语义（术语）问题外，CCA的分类也存在疑问，因为通过对主要患者群体的分析发现，软骨钙质沉着症或假痛风（18, 19）或二水焦磷酸钙晶体沉积病（17）的情况各不相同。在我们的论文中，我们阐述了对CCA原发性形式的看法，并提出了基于对我们患者群体20多年观察得出的分类特征，特别是基于对临床和X线表现开始情况以及疾病进一步发展的评估。