Calcium pyrophosphate deposition disease
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Synopsis
CPPD disease usually affects patients 60 years and older and can have overlap with osteoarthritis. It affects women and men equally. Metabolic derangements such as hypophosphatemia, hypomagnesemia, and hypercalcemia increase the risk of CPPD. As such, conditions that predispose to metabolic derangements are associated with CPPD, such as hyperparathyroidism, hypothyroidism, Gitelman syndrome, hemochromatosis, and loop diuretic use. Similarly, inflammatory conditions of the joint can predispose to CPPD such as acute illness or joint trauma during the postoperative period, rheumatoid arthritis, and osteoarthritis.
While acute CPPD is more common and is often mistaken for gout, the less common chronic form of CPPD (<5% of cases of CPPD) can be mistaken for rheumatoid arthritis. Chronic CPPD often involves multiple joints, commonly involving the small peripheral joints of the arms and legs, often symmetrically. Inflammation can last for months but, unlike rheumatoid arthritis, inflammation of the affected joints can wax and wane independently of one another.
If the disease occurs in patients younger than 60, a familial history should be elicited as there are certain genetic associations that can cause earlier onset of disease.
The pathophysiology of pseudogout is not well understood, but CPP crystals forming in the cartilage is the first step in the disease process. Deposition of the CPP crystals affects the balance toward the production of pro-destructive prostaglandins and metalloproteinases, which destroy the chondrocytes and synoviocytes, cells that are important for the structural framework of cartilage and synovium.
Codes
ICD10CM:E83.59 – Other disorders of calcium metabolism
SNOMEDCT:
239832006 – Calcium Pyrophosphate Deposition Disease
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Differential Diagnosis & Pitfalls
- Gout
- Osteoarthritis
- Rheumatoid arthritis
- Septic arthritis
- Lyme disease
- Trauma
- Polymyalgia rheumatica
- Neuropathic arthropathy (Charcot joints)
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.Subscription Required
References
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Last Reviewed:06/12/2017
Last Updated:06/14/2017
Last Updated:06/14/2017
Patient Information for Calcium pyrophosphate deposition disease
Overview
Calcium pyrophosphate deposition disease (CPDD), commonly known as pseudogout due to its similarity to gout, is an arthritis in the joints characterized by episodes of sudden and painful swelling.It is caused by an abnormal collection of calcium pyrophosphate in the joints. Both gout and CPDD result from crystal deposits within a joint but differ by the type of crystal formed. It most often targets the knee joint, but can also affect wrist or ankle joints.
Who’s At Risk
Risk factors for CPDD include old age, prior injury or joint surgery, family history of joint problems, thyroid and parathyroid abnormalities, or other medical disorders.Signs & Symptoms
The symptoms of benign CPDD include:- Swollen joints
- Warm joints
- Severe pain in joints
Self-Care Guidelines
Over-the-counter medication such as ibuprofen (Advil, Motrin) can help relieve symptoms. Resting and icing the joints can help reduce pain and swelling.When to Seek Medical Care
Seek medical care if your joint pain is sudden and severe, joint appears swollen and warm, or if symptoms do not subside with minimal self-care listed above.Treatments
You may be referred to a specialist (rheumatologist) for evaluation.If over-the-counter medications are not helping, your health care provider may prescribe:
- Nonsteroidal anti-inflammatory drugs (NSAIDs) in prescription dose such as naproxen (Anaprox, Naprosyn) and indomethacin (Indocin). You will be monitored to assure that this medication is not diminishing kidney function or leading to stomach bleeding
- Colchicine can be used for prevention of CPDD attacks
- Corticosteroids such as prednisone may be administered if you cannot take nonsteroidal anti-inflammatory drugs or colchocine
Joint drainage is another method to reduce pain from CPDD. This is done by inserting a needle into your joint to remove some fluid and crystals that cause the pain. You may also receive an injection to reduce pain and inflammation in the joint.
Calcium pyrophosphate deposition disease