• Information
  1. Tumor Related Arthritis
  2. Amyloid Associated Arthritis
  3. Pancreatic Panniculitis
  HEMOCHROMATOSIS
  1. Presenting Symptoms
  2. Laboratory
  3. Treatment

• ARTHRITIS WITH HYPERPIGMENTATION
  1. Common Causes
  2. Endocrine
  3. Uncommon
  4. Introduction
  5. Diagnosis
  6. Associated Disorders
  7. Complications
  8. Treatment

A. Tumor Related Arthritis

1. Paraneoplastic syndromes - usually polyarticular
2. Leukocyte Related
   1. Multiple Myeloma
   2. Lymphoma (usually B cell)
   3. Leukemia - direct infiltration, usually with monocytic or myelomonocytic tumors [6]
   4. Myelodysplastic Syndromes (up to 10% of cases)
   5. Angioimmunoblastic Lymphadenopathy (AILD)
   6. Usually polyarticular
3. AILD [1]
   1. Arthritis: symmetric, polyarticular and nonerosive
   2. Skin Rash
   3. Fevers, Myalgias, Systemic Symptoms - sudden onset
   4. Most common in older individuals
   5. Polyclonal gammopathy
4. Tumors of Joints
   1. Pigmented Vilonodular Synovitis (PVNS)
   2. Synovial Chondromatosis - focal metaplastic growths withnormal cartilage
   3. Hemangiomas
   4. Synovial Sarcoma (Malignant Synovioma) - usually adjacent to joint within tendon
5. Pigmented Vilonodular Synovitis (PVNS)
   1. Proliferation of synovial cells, usually single joint (knee most common)
   2. Young adults, M=F
   3. Joint effusion shows hemosiderin granules with lipid depositis (brownish color)
   4. Synovial biopsy shows laarge fingerlike villi, multinucleated giant cells
   5. Treated by complete synovectomy
   6. Irradiation may be used when surgery contraindicated

B. Amyloid Associated Arthritis

1. Occurrance
   1. Primary Amyloid
   2. Amyoidosis secondary to plasma cell dyscrasia
2. Symptoms
   1. Morning stiffness
   2. Nodules
   3. Affects multiple joints in a symmetric pattern
   4. Signs very similar to Rheumatoid Arthritis
3. Plasma Cell Dyscrasias and Amyloid Associated Arthritis [2]
   1. Most cases involving multiple myeloma, usually with L Chains (which form amyloid)
   2. Macroglobulinemia very uncommonly associated with disease
   3. Monoarticular or polyarticular
   4. May be inflammatory
   5. Treat symptoms with NSAIDs; consider glucocorticoids
4. Dialysis Associated Arthritis
   1. ß2-microglobulin associated disease
   2. Usually related to duration of dialysis and due to ß2-microglobulin accumulation
   3. NSAIDs rarely effective; low dose glucorticoids (10-20mg/d prednisone) effective
   4. Renal transplantation results in rapid decrease in ß2-microglbulin levels
   5. Renal transplantation did not reverse radiographic changes [3]
   6. However, joint symptoms decreased, probably due to post-transplant glucocorticoids

C. Pancreatic Panniculitis

1. Pathophysiology
   1. Severe inflammation with destruction of dermal fat cells
   2. Usually associated with severe pancreatitis
   3. Inflammation of synovial lining with fat destruction as well
2. Diagnosis
   1. Appearance of joint fluid - lipid laden
   2. Presence of severe pancreatitis
   3. Usually self limited; very painful
3. Treatment
   1. Treatment with NSAIDs, tylenol,
   2. Opiates may be required; avoid morphine because it can cause sphincter of Odi spasm

1. Chronic liver function anomalies - AST (SGOT) and ALT (SGPT) elevations
2. May cause cirrhosis; increased risk of hepatoma
3. Diabetes Mellitus - often called "Bronz Diabetes"
4. Arthritis
   1. Predisposition for MCP's, especially 2nd and 3rd
   2. Radiographs show "hook-like" appearance of MCP's, osteophytes, osteoporosis
   3. Cystic lesions with sclerotic walls, joint space narrowing
   4. Association with CPPD also occurs leads to larger joints, often with chondrocalcinosis
5. Cardiovascular Disease - conduction system, infiltrative cardiomyopathy
6. Hypogonadism

B. Laboratory

1. High Serum Iron Levels
2. Ferritin >1000 (often 3000-5000)
3. Very low transferrin saturation
4. Hyperglycemia may occur due to pancreatic disease

C. Treatment

1. Phlebotomy is major therapy
2. Oral Iron Chelators now available
3. Therapy of arthritis - NSAIDs first line
4. The condition of the underlying disease may not correlate with arthritis

1. Whipple's Disease
2. Primary Biliary Cirrhosis
3. Hemochromatosis

B. Endocrine

1. Cushing's Syndrome
2. Addison's Disease
3. Probably related to ACTH production

C. Uncommon

1. Ochranosis
2. Diffuse Scleroderma
3. POEMS Syndrome
4. Systemic Lupus Erythematosus

1. Formerly called Forestier's Disease
2. Common disorder amongst older adults
3. Hyperostosis syndrome

B. Diagnosis

1. Based solely on radiographic (Resnick) criteria:
   1. Ossification of anterior longitudinal ligament of the spine
   2. Ossification of various extraspinal ligmaents
2. Serum matrix GIa protein may be a marker of hyperostotic syndromes

C. Associated Disorders

1. Increased body mass index
2. Elevated serum uric acid levels
3. Increased incidence of diabetes mellitus type 2

D. Complications

1. Dysphagia due to large anterior osteophytes of C-spine may occur - most common
2. Stridor, dyspnea
3. Myelopathy associated with ossification of posterior longitudinal ligament
4. Atlanto-axial pseudoarthrosis or subluxation can also occur (with myelopathy)
5. Thoracic outlet syndrome is uncommon
6. Endoscopic and/or intubation difficulties can occur

E. Treatment

1. None specific
2. Surgery may be required to resect osteophytes
3. Pain managment

References

1. Gravallese EM, Winalski CS, Longtine J, Helfgott SM. 1994. Arthritis Rheum. 37(7):1087
2. Donnelly S, Bourne JT, Levison DA, et al. 1993. Br J Rheumatol. 32:1004
3. Bardin T, Lebail-Darne JL, Zingraff J, et al. 1995. Am J Med. 99(3):243
4. Sarzi-Puttini P and Atzeni F. 2004. Curr Opin Rheumatol. 16(3):287
5. Mader R. 2002. Semin Arthritis Rheum. 32(2):130
6. Schwarzberg AB, Alexander CB, DeAngelo DJ, Helfgott SM. 2008. NEJM. 358(23):2496 (Case Discussion)