[First classification criteria for diseases caused by calcium pyrophosphate deposition (CPPD)-Translation, explanation and assessment]. / Erste Klassifikationskriterien für durch Kalziumpyrophosphatablagerungen verursachte Erkrankungen ­ Übersetzung, Erläuterung und Bewertung.

AIM: For diseases caused by calcium pyrophosphate deposition (CPPD), validated classification criteria were previously lacking. In this article the recently developed and validated classification criteria are translated, explained, and assessed. METHODS: In recent years a multinational research group developed classification criteria for CPPD disease with the support by the European Alliance of Associations for Rheumatology (EULAR) and the American College of Rheumatology (ACR), following an established method. The developed criteria were finally validated in an independent cohort. The translation and annotation of the new first classification criteria were carried out in an iterative procedure in consensus with the authors. RESULTS: The presence of a crowned dens syndrome or calcium pyrophosphate crystals in the synovial fluid in patients with pain, swelling or sensitivity of the joints (entry criterion) is sufficient for the classification as CPPD disease, where the symptoms cannot be completely explained by another rheumatic disease (exclusion criterion). If these symptoms are not present, a count of more than 56 points based on weighted criteria comprised of clinical features and the results of laboratory and imaging investigations can be included for classification as a CPPD disease. These criteria had a sensitivity of 92.2% and a specificity of 87.9% in the derivation cohorts (190 CPPD cases and 148 mimics), whereas the sensitivity was 99.2% and the specificity 92.5% in the validation cohorts (251 CPPD cases and 162 mimics). CONCLUSION: The ACR/EULAR classification criteria 2023 of a CPPD disease will facilitate clinical research in this field. The use in the clinical routine will show how practical the criteria are.

Cauda Equina Syndrome Caused by Pseudogout Involving the Lumbar Intervertebral Disc

Calcium pyrophosphate dihydrate (CPPD) deposition disease, also known as pseudogout, is a disease that causes inflammatory arthropathy in peripheral joints, however, symptomatic involvement of the intervertebral disc is uncommon. Herein, we describe a 59-yr-old patient who presented with cauda equina syndrome. Magnetic resonance imaging of the patient showed an epidural mass-like lesion at the disc space of L4-L5, which was compressing the thecal sac. Biopsy of the intervertebral disc and epidural mass-like lesion was determined to be CPPD deposits. We reviewed previously reported cases of pseudogout involving the lumbar intervertebral disc and discuss the pathogenesis and treatment of the disease.

Condrocalcinosis e hipomagnesemia: evolución clinicorradiológica / Chondrocalcinosis and hypomagnesemia: clinical and radiological progression

Entre las causas secundarias infrecuentes de la enfermedad por depósitos de cristales de pirofosfato cálcico se incluye la hipomagnesemia. Se presentan 2 casos de pacientes jóvenes con ataques repetidos de monoartritis en rodillas con condrocalcinosis articular e hipomagnesemia. Tras seguimiento clinicorradiológico y tratamiento con lactato de magnesio durante al menos 5 años, los pacientes no han presentado nuevos ataques de seudogota en rodillas. Se discute la evolución clinicorradiológica de las rodillas afectadas en ambos pacientes; destaca el mayor deterioro radiológico en sus rodillas del paciente con inicio más precoz y antecedentes familiares de condrocalcinosis articular, a pesar de seguir clínicamente asintomático (AU)

Hypomagnesemia is a rare secundary metabolic disorder associated with calcium pyrophosphate dihydrate cristal deposition disease in joint structures and may cause asymptomatic chondrocalcinosis (linear calcification of cartilage), pseudogout, and chronic arthropathy. We report 2 young men with relapsing acute knee monoarthritis with chondrocalcinosis and hypomagnesemia. After follow-up clinical and radiological events at least for 5 years and treatment with magnesium lactate, these patients have not shown new pseudogout attacks. We discuss knee radiological evolution in both patients, outstanding major knee radiological deterioration in the patient with early symptoms and a familial chondrocalcinosis association, in spite of clinical asymptomatic status (AU)

Idiopathic Calcium Pyrophosphate Dihydrate (CPPD) Crystal Deposition Disease in a Young Male Patient: A Case Report

Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is a disease of the elderly and extremely rare in young individuals. If young people develop CPPD crystal deposition disease, it may be associated with metabolic diseases such as hemochromatosis, hyperparathyroidism, hypophosphatasia, hypomagnesemia, Wilson's disease, hypothyroidism, gout, acromegaly, and X-linked hypophosphatemic rickets. Therefore, in young-onset polyarticular CPPD crystal deposition disease, investigation for predisposing metabolic conditions is warranted. We report a case of a young male patient with idiopathic CPPD crystal deposition disease, who did not have any evidences of metabolic diseases after thorough evaluations. As far as we know, this is the first report of a young male patient presented with idiopathic CPPD crystal deposition disease.

Condrocalcinosis articular / Articular chondrocalcinosis

El trabajo está basado en una muestra de 37 observaciones de condracalcinosis articular, seleccionadas de acuerdo a los criterios propuestos por Mac Carty. Se comienza por fijar la situación nosológica de la afección, estableciendo el concepto de enfermedades por depósito de microcristales. Se informa sobre la frecuencia de la condrocalcinosis articular en nuestro medio, su incidencia etaria y por sexo. Se analizan los diferentes patrones clínicos, se hace referencia a las imágenes radiológicas más frecuentes y se comentan los hallazgos de laboratorio. Se intenta una síntesis de los supuestos mecanismos fisiopatológicos planteados en la actualidad. Finalmente se destaca la existencia de formas inhabituales, especialmente de arteriopatías destructivas, y se esbozan directivas terapéuticas