What is the management for Calcium Pyrophosphate Deposition (CPPD) disease?

Management of Calcium Pyrophosphate Deposition (CPPD) Disease

The optimal treatment of CPPD requires tailored pharmacological and non-pharmacological approaches based on clinical presentation (acute vs. chronic), patient factors (age, comorbidities), and presence of any underlying metabolic disorders. 1

Clinical Presentations and Treatment Approach

Asymptomatic CPPD/Chondrocalcinosis

• No treatment is required for asymptomatic chondrocalcinosis 1, 2
• Currently, no treatment modifies CPP crystal formation or dissolution 2, 3

Acute CPP Crystal Arthritis (Pseudogout)

First-line treatments:

• Application of ice or cool packs, temporary rest, joint aspiration, and intra-articular injection of long-acting glucocorticosteroids (GCS) 1, 4
• For monoarticular or oligoarticular attacks, joint aspiration with intra-articular GCS injection is highly effective and safe 1, 4

Systemic treatments when intra-articular injection is not feasible:

• Oral NSAIDs (with gastroprotection if indicated) 1
• Low-dose oral colchicine (0.5 mg up to 3-4 times daily, with or without an initial dose of 1 mg) 1, 4
  • Most effective when started within 12 hours of symptom onset 4
  • High-dose regimens cause significant side effects and should be avoided 4
• A short tapering course of oral GCS, parenteral GCS, or ACTH for patients with contraindications to NSAIDs or colchicine 1
  • Oral prednisone/prednisolone 30-35 mg daily for 3-5 days 4

Important considerations:

• Treatment should be initiated as early as possible for optimal effectiveness 4
• NSAIDs and colchicine use is often limited by toxicity and comorbidities, especially in older patients 1
• Parenteral GCS may provide quicker pain control than NSAIDs 1
• For refractory cases, anakinra (IL-1 receptor antagonist) can be effective 5, 6

Prophylaxis Against Recurrent Attacks

• Low-dose oral colchicine (0.5-1 mg daily) 1
• Low-dose oral NSAIDs with gastroprotection if indicated 1

Chronic CPP Crystal Inflammatory Arthritis

• First-line: Oral NSAIDs (with gastroprotection) and/or colchicine (0.5-1.0 mg daily) 1, 7
• Second-line: Low-dose corticosteroid, methotrexate (5-10 mg/week), or hydroxychloroquine 1, 7
• For refractory cases: Tocilizumab has shown efficacy 5, 6
• Intra-articular radiocolloid (yttrium-90) injection may provide significant pain improvement in knee OA with CPPD 7

OA with CPPD

• Management objectives and treatment options are the same as for OA without CPPD 1, 7
• Standard OA management principles including education, physical therapy, and joint protection strategies 7
• Avoid intra-articular high molecular weight hyaluronan as it might induce acute attacks 7

Management of Associated Conditions

• If detected, treat associated conditions such as hyperparathyroidism, hemochromatosis, or hypomagnesemia 1, 2
• Early disease (onset before age 60) requires evaluation for these metabolic conditions 2

Common Pitfalls and Caveats

• Failing to start treatment early significantly reduces effectiveness 4
• Using traditional high-dose colchicine regimens leads to marked side effects 4
• Intravenous colchicine should be avoided due to high risk of serious toxicity 1
• Carefully consider comorbidities and age when selecting treatments, as CPPD predominantly affects older patients 7
• Monitor for side effects of long-term treatments, particularly with NSAIDs, colchicine, and methotrexate 7

Treatment Algorithm for Acute Attacks

1. For monoarticular/oligoarticular attacks affecting accessible joints:

   • Joint aspiration followed by intra-articular GCS injection 1, 4

2. For polyarticular attacks or inaccessible joints:

   • If within 12 hours of symptom onset: Colchicine 1.2 mg followed by 0.6 mg one hour later 4
   • If beyond 12 hours or colchicine contraindicated: NSAIDs at full dose or oral prednisone 30-35 mg daily for 3-5 days 4
   • For severe attacks: Consider combination therapy 4