What is the recommended management strategy for a patient with calcium pyrophosphate deposition disease (CPPD)?

Management of Calcium Pyrophosphate Deposition Disease (CPPD)

For acute CPP crystal arthritis, joint aspiration combined with intra-articular long-acting glucocorticosteroid injection is the optimal first-line treatment and is often sufficient alone. 1, 2

Treatment Algorithm by Clinical Phenotype

Acute CPP Crystal Arthritis (Pseudogout)

First-line approach:

• Ice or cool packs applied to affected joint 1
• Temporary rest of the joint 1
• Joint aspiration to remove inflammatory fluid 1
• Intra-articular injection of long-acting glucocorticosteroid 1, 2

This combination alone is sufficient for many patients and has a strength of recommendation of 95% 1.

Second-line systemic options when intra-articular injection is not feasible:

• Oral NSAIDs with gastroprotective treatment (especially critical in elderly patients) 1, 2
• Low-dose oral colchicine: 0.5 mg up to 3-4 times daily (avoid traditional high-dose regimens due to marked toxicity) 1, 2
• Short tapering course of oral glucocorticosteroids 1
• Parenteral glucocorticosteroids or ACTH for polyarticular attacks 1

The evidence shows that parenteral glucocorticosteroids (7 mg betamethasone IM or 125 mg methylprednisolone IV) achieve faster pain control than NSAIDs on day 1 (NNT=3), though differences disappear by day 3 1.

Refractory cases:

• Anakinra (IL-1 receptor antagonist) for acute flares that fail standard treatment 3

Prophylaxis Against Recurrent Acute Attacks

Recommended regimens:

• Low-dose oral colchicine: 0.5-1.0 mg daily 1, 2
• Low-dose oral NSAIDs with gastroprotection 1, 2

Strength of recommendation is 81% for prophylactic therapy 1.

Chronic CPP Crystal Inflammatory Arthritis

Treatment hierarchy (in order of preference):

1. Oral NSAIDs with gastroprotection and/or low-dose colchicine (0.5-1.0 mg daily) 1, 2
2. Hydroxychloroquine (preferred second-line agent) 2, 3
3. Low-dose corticosteroids 1
4. Methotrexate 1
5. Tocilizumab for refractory cases 3

The strength of recommendation for this hierarchy is 79% 1.

Osteoarthritis with CPPD

Management approach:

• Treat identically to OA without CPPD using standard evidence-based OA guidelines 1, 2
• Critical exception: Avoid intra-articular high molecular weight hyaluronan 2

Strength of recommendation is 84% 1.

Asymptomatic Chondrocalcinosis

No treatment required - this is typically an age-related finding and does not warrant intervention 1, 2. Strength of recommendation is 90% 1.

Evaluation for Underlying Metabolic Disorders

Screen for and treat if detected:

• Primary hyperparathyroidism 1, 4
• Hemochromatosis 1, 4
• Hypomagnesemia 1, 4

Strength of recommendation for treating associated conditions is 89% 1.

Special consideration for diuretic use:

• Furosemide causes magnesium depletion, which promotes CPP crystal formation 5
• Consider alternative diuretics with less impact on magnesium levels when possible 5
• If furosemide is necessary, supplement magnesium 5

Critical Pitfalls to Avoid

• Never use traditional high-dose colchicine regimens - marked side effects limit use 2
• Never use intravenous colchicine - serious toxicity risk 2
• Never use intra-articular hyaluronan in CPPD patients 2
• Always provide gastroprotection with NSAIDs in older patients - CPPD predominantly affects elderly with higher GI bleeding risk 1, 2
• Do not treat asymptomatic chondrocalcinosis found incidentally on imaging 1, 2

Key Limitations in CPPD Management

Currently, no disease-modifying therapies exist that eliminate CPP crystals or reduce articular calcification 1, 3. All treatments are symptomatic and aimed at controlling inflammation rather than crystal dissolution 1.