Condrocalcinosa

Condrocalcinoza - storage disease calcium pyrophosphate is a rheumatologic disorder with varied clinical manifestations due to precipitation of calcium pyrophosphate in connective tissues. It's known as alternative names that specify certain clinical or radiographic descriptions, although neither is synonymous with storage disease calcium pyrophosphate.Pseudogout refers to the evidence of acute clinical sinovitei joints red, swollen and sensitive that are similar to arthritis of gout.Condrocalcinoza refers to the radiographic appearance of hyaline cartilage calcifications or fibrocartilajului. Arthropathy with pyrophosphates is a term that refers to both the above conditions.Statistics is the most affected knee joint.
Storage disease calcium pyrophosphate crystals of mineral deposit consists of soft tissues. It is found in high concentrations in hyaline cartilage, synovial tissue, capsule, meniscus, ligament yellow, soft tissues of the hand and rare fibrocartilajul temporomandibular joint. Polyarticular arthritis is monoarticular although initially starts. Although in theory any joint can be affected no preferential localization. In rare cases pseudogout can affect the spinal canal and cause myelopathy.
Condrocalcinozei exact pathology is unknown, although cleavage increased adenosine triphosphate (energy molecule necessary for most anmalelor) may increase the level of pyrophosphate in the joints and is considered a method of crystal growth. There is some research that suggests the involvement of genes with a role in inorganic phosphate transport.
Patients usually present with inflammation in one or more joints with pain. Hyperparathyroidism, hemochromatosis, and hypophosphatemia associated with renal osteodistrophy condrocalcinoza. Other risk factors are condrocalcinoza Wilson's disease and osteochondritis.
Treatment for asymptomatic form of the disease does not prevent joint destruction. Acute pseudogout is recommended for intra-articular corticosteroid injections, systemic corticosteroids, NSAIDs or colchicine. Surgical removal of calcifications still remains an experimental procedure.
Pathogenesis condrocalcinozeiThe exact physiological dysfunction condrocalcinoza is not clear, but studies involving condrocitul and surrounding matrix as responsible agents. Some chemical and physical events determine the activation of a cascade that evolves into chondrocyte hypertrophy and degeneration. Escape into the surrounding intercellular matrix material and alter the binding of calcium effect of matrix proteoglycans. Calcium pyrophosphate crystals grow from the matrix of hypertrophic chondrocytes adjacent to those affected. Rupture of collagen fibers has been proposed to be the source of inorganic pyrophosphate.
Storage disease calcium pyrophosphate is the result of a cascading inflammatory response to the storage crystal. Enzyme abnormalities or allow the formation of pyrophosphate in excess of saturation, which precipitated especially hyaline or fibrous cartilage. People with this disorder may have more than one type of crystals (hydroxyapatite and pyrophosphate). The disease can be divided into two varieties: primary and secondary. Refers to secondary disease associated with rheumatoid arthritis spondiloartropatia. Distribution of disease in people with secondary form is identical to that seen in primary form. Primary form can be divided in turn into family variants, metabolic and idiopathic. The joint damage is different between them. Variety abolii family tends to begin earlier in life than one idiopathic.Metabolic Causes and associations include: hyperparathyroidism, haemochromatosis, hypomagnesaemia, gout, ochronosis, Wilson disease, acromegaly, Paget's disease and sidnromul Gitelman.
Among varieties metabolic impairment hemochromatosis causes more severe the hand wrist, knee, ankle and metacarpals, while ochronosis affects especially the shoulder, hip, knee and spine.Hemochromatosis is a disorder of iron metabolism that accumulate and destroy body tissues, including joints. Wilson's disease consists of abnormal accumulation of copper. Ochronosis is another storage disease in homogentisinic acid accumulates as a result of the metabolising enzyme deficiency. Gitelman syndrome is a variant of Bartter-sidnromului autosomal recessive renal tubular disease. Varieties of metabolic storage disease is genetic calcium pyrophosphate.
Signs and symptoms of condrocalcinozaCondrocalcinoza or storage disease, a variant of calcium pyrophosphate arthritis. It is a chemical that manifest error by at least four associated clinical entati. Condrocalcinoza is described as soft tissue impregnation with calcium. The term is sometimes used synonymously with storage disease Calcium pyrophosphate, but technically refers to the visible presence of calcification in soft tissue imaging studies.
Condrocalcinozei manifestations include four main distinct entities:Pseudogout:Presentation appears to be similar to acute gout. Technically it is called pseudogout although the term is used synonymously with calcium pyrophosphate storage disease. Gout can be distinguished by shape crystals of pseudogout: gout crystals (sodium ugly) are in the form of needles and not birefringence, while crystals of pseudogout (calcium pyrophosphate) are diamond-shaped and have positive birefringence. Advanced electron microscopy techniques can be used to finalize the diagnosis.
Pseudogout affecting a small percentage of people condrocalcinoza-25%. Onset is usually monoarticular or pauciarticular, and is preceded by injury or surgery in the area.Pseudogout was identified after adenoma excision parotiroidian.Onset is aggressive, reaches a peak within a few hours and is manifested by pain, swelling, local heat and redness. Fever is present in half of patients with pseudogout. The knee is affected most frequently. Shoulder, elbow, ankle and joint first metacarpofalangiana are commonly affected. Evolution includes natural spontaneous resolution within a few days or weeks at most.Treatment speeds recovery.
The disease is characterized by acute oligoarticular arthritis or monoarticulara. Clinical manifestations are similar to gout. Shows acute swelling and pain but not so intense. Pseudogout can be precipitated by medical conditions such as myocardial infarction, congestive heart failure, cerebrovascular accident, or can occur after surgery. Trauma can also be a trigger. Occasionally, pseudogout may present as a syndrome pseudoseptic acute arthritis, fever and leukocytosis.Pseudogout tofacee:Mineral material can be stored in large reservoirs producing a pseudotumor. This can be massive-loaded with consequences. I am discreet painful. Lesions are found in the temporomandibular joint, sternoclaviculara, transverse ligament of C1, metatarsophalangeal joints, spinal facet joints. Impaired spinal nerve is associated with incarceration and symptomatic spinal stenosis requiring surgical decompression.Storage of calcium pyrophosphate family:A family pattern occurs at a young age, usually the third decade of life. Tends to be more aggressive with more negative long-term prognosis. Longevity satisfactory joint function is reduced in these individuals. Genetic studies involving a gene responsible for autosomal dominant inheritance mode. Gene mutation cause high levels of intracellular inorganic pyrophosphate. Low levels of extracellular inorganic pyrophosphate allows storage of hydroxyapatite in articular hyaline cartilage and fibrocartilaj.Osteoarthritis:The most common is osteoarthritis condrocalcinozei presentation.Symptoms are similar to those of the typical patient with osteoarthritis. Note that this condrocalcinozei worsens with age.
Other variants of condrocalcinozei include:Pseudosteoartrita:Often affects metacarpophalangeal joints, wrists, elbows and shoulders, joints affected by osteoarthritis are not primary. Most commonly affects the knee. This variant of the disease include 50% of cases. Approximately 50% of patients and associated with pseudogout.Bone spurs are shaped hook radiological elements common in these patients and found the initial and second metacarpal heads.This condrocalcinozei the radiograph is common.Arthritis pseudoreumatoida:This model of the disease is found in approximately 5% of patients with storage disease and calcium pyrophosphate is associated with inflammation of the metacarpophalangeal joints symmetrically.Clinic patients accuse morning stiffness and swelling. Erythrocyte sedimentation rate is high. Late age of onset, rheumatoid factor, and this lack condrocalcinozei help differentiate from rheumatoid arthritis.Pseudoneuropatica joint:Neuropaticlike arthropathy is seen in less than 5% of patients.Most often affects the knee. It is a severe destructive condition.Unlike true neuropathic arthropathy presents no clear basis of neurological condition. This condrocalcinozei support the implementation of diagnosis.