Case of the Month February 2014, page 2

ANSWER:  E

EXPLANATION

For a full-size image, please click Wrist X-ray 2 .



IMAGE:  PA radiograph of the right wrist shows subchondral cyst formation of the distal ulna (green arrow) as well as amorphous chondrocalcinosis within the triangular fibro-cartilage complexes (red arrow).  There is narrowing of the radioscaphoid joint space and a SLAC wrist deformity with scapholunate widening (blue arrow) and with excavation of the radius by the scaphoid.

DISCUSSION:  Calcium pyrophosphate deposition (CPPD) is a relatively common disease that occurs predominately in the elderly population and is characterized by the deposition of pyrophosphate crystals within the articular tissues; most commonly fibrocartilage and hyaline cartilage.  The general features of CPPD are chondrocalcinosis, atypical distribution of osteoarthritis, and subchondral cyst formation.  CPPD is one of many causes of chondrocalcinosis.  It is not synonymous with chondrocalcinosis and not the only cause of soft tissue calcification.  The most common locations of CPPD is the knee, wrist, hand and shoulders.

The terminology for CPPD is often a point of confusion.  In recent literature, it was agreed that ‘CPPD’ should be the umbrella term that includes asymptomatic CPPD, acute calcium pyrophosphate (CPP) crystal arthritis, osteoarthritis (OA) with CPPD, and chronic CPP crystal inflammatory arthritis.  These are all different phenotypical manifestations of CPPD.  A commonly used older term ‘pseudogout’ refers to the similarity of clinical presentation of CPPD to gout.  This is only one of the possible clinical presentations of CPPD.  

When CPPD occurs in the wrist there is a typical constellation of radiographic findings.  The most common finding is amorphous calcifications within the triangular fibrocartilage complex (TFCC) of the wrist, radiocarpal narrowing and in advanced cases scapholunate advanced collapse.





IMAGE

From left to right:  Asymptomatic CPPD:  The patient can have crystals in joint in laboratory test, but be clinically asymptomatic and radiographically normal. In this subtype of CPPD the patient may not event have chondrocalcinosis.  Osteoarthritis (OA) with CPPD:  Particularly targets knees with chronic symptoms and/or acute attacks of crystal-induced inflammation.  When compared to typical age related OA It may associate with more inflammatory symptoms and signs, an atypical distribution (eg, radiocarpal or midcarpal, glenohumeral, hindfoot or midfoot involvement) and prominent cyst and osteophyte formation on radiographs. Acute CPP Crystal Arthritis: The rapid development of severe joint pain, swelling and tenderness that reaches its maximum within 6–24 h, especially with overlying erythema, is highly suggestive of acute crystal inflammation though not specific for acute CPP crystal arthritis.  Before this new nomenclature paradigm this would have been called pseudogout.  Chronic CPP Crystal Inflammatory Arthritis:  Presents as chronic oligoarthritis or polyarthritis with inflammatory symptoms and signs and occasional systemic upset (with elevation of CRP and ESR); superimposed flares with characteristics of crystal inflammation support this diagnosis. It should be considered in the differential diagnosis of rheumatoid arthritis and other chronic inflammatory joint diseases in older adults. Radiographs may assist diagnosis, but the diagnosis should be crystal proven.


References


1. Wise CM: Crystal-associated arthritis in the elderly. Rheum Dis Clin North Am. 33(1):33-55, 2007
2. Zaka R et al: Genetics of chondrocalcinosis. Osteoarthritis Cartilage. 13(9):745-50, 2005
3. Marsot-Dupuch K et al: Massive calcium pyrophosphate dihydrate crystal deposition disease: a cause of pain of the temporomandibular joint. AJNR Am J Neuroradiol. 25(5):876-9, 2004
4. Zhang W, Doherty M, Bardin T, et al. European League Against Rheumatism recommendations for calcium pyrophosphate deposition. Part I: terminology and diagnosis. Ann Rheum Dis. 2011;70(4):563-70. Epub 2011 Jan 7.

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