9: Miscellaneous arthropathies

Miscellaneous Arthropathies


Robin Geletka1, Karen Law2


1Actelion Pharmaceuticals Ltd, San Francisco, CA, USA


2Emory University School of Medicine, Atlanta, GA, USA


Introduction


Arthritis can often be a manifestation of underlying systemic disease. Arthritis from malignancy and hematologic disorders, endocrinopathies, and infectious disease must be considered in the differential list in patients presenting with arthritis. Understanding the characteristics of these disorders is critical to making the diagnosis of the underlying disease.


Arthritis from Malignancy and Hematologic Disorders


Paraneoplastic Syndromes



  • May coincide with, follow, or precede onset of malignancy
  • Treatment involves therapy directed towards primary malignancy
  • Hypertrophic osteoarthropathy

    • Characterized by proliferation of bone at distal extremities
    • Associated with clubbing, periosteal proliferation of tubular bones, and joint effusions
    • May be associated with lung malignancy; in these cases resection of malignancy results in resolution of arthropathy
    • Synovial WBC count is non-inflammatory
    • Pain involves both joint and bone

  • Carcinomatous polyarthritis

    • Inflammatory seronegative polyarthritis
    • Late age of onset and commonly spares hands and wrists
    • Typically there is no evidence of direct metastasis
    • Found more commonly in women with breast cancer and men with lung cancer

  • Erythromelalgia

    • Intense pain, redness, and warmth in the digits, palms, and soles
    • Most commonly found in patients with polycythemia vera or essential thrombocytosis, though it may also be idiopathic
    • Responds to aspirin, dosed up to 650 mg TID

Direct Tumor Invasion Causing Arthritis



  • Leukemic arthritis

    • Most common in acute lymphocytic leukemia in children
    • Asymmetric polyarthritis of large joints
    • Severe joint and bone pain
    • Leukemic cells can be found in synovial fluid or synovial tissue
    • Associated with an increased frequency of gout and septic arthritis

  • Lymphomatous arthritis

    • A rare mono- or polyarthritis
    • Affected patients also experience increased frequency of gout and septic arthritis

  • Multiple myeloma

    • Malignant plasma cell tumor often associated with axial bone pain
    • Arthritis is rare
    • Associated with AL amyloid arthropathy

      • Symmetric, non-inflammatory arthritis
      • Typically affects shoulders, wrists, and knees
      • Shoulder involvement is called “shoulder pad sign”
      • Diagnosis made by bone marrow biopsy, presence of M protein, and biopsy of synovium showing amyloid

  • Metastatic carcinomatous arthritis

    • Lung cancer is the most common cause
    • Manifests as a non-inflammatory monoarthritis of large joints

Hematologic Disorders



  • Hemophilia A and B

    • Recurrent hemarthrosis is the most common manifestation
    • May occur spontaneously or following trauma
    • Over time, patients may develop a chronic arthropathy thought to be secondary to repeated bleeding episodes and deposition of iron in the synovium
    • Associated with osteonecrosis (AVN) of the hip
    • Treatment involves factor replacement, immobilizaton, and ice
    • Patients have a much higher risk of septic arthritis; a low threshold for joint aspiration is recommended if infection is suspected

  • Sickle cell disease

    • Sickle cell arthropathy is caused by microvascular ischemia, usually in large joints
    • Associated with osteonecrosis most often affecting the hip
    • Vaso-occlusive crises may result in dactylitis, which may be the presenting symptom of sickle cell disease in children

Endocrinopathies


Diabetes Mellitus



  • Diabetes mellitus is associated with a higher risk of multiple musculoskeletal manifestations, most of which are associated with poor glycemic control
  • Diabetic cheiroarthropathy

    • A disorder involving the soft tissues of the hands and fingers resulting in waxy skin thickening and tightening and joint contractures
    • Classic finding called the “prayer sign,” in which the palms and fingers do not touch when the hands are pressed together
    • Pathogenesis is believed to be related to excess glycosylation of collagen in the skin and joint tissues

  • Adhesive capsulitis

    • A condition causing limitation in shoulder motion often called “frozen shoulder”
    • Treatment can involve a combination of NSAIDs, steroid injections, and physical therapy

  • Dupuytren’s contracture

    • An abnormality of the collagen in the flexor tendons of the hand, causing shortening and nodularity
    • Patients affected have an inability to fully flex or extend affected fingers
    • Treatment includes surgical removal of collagen followed by physical therapy
    • Newer treatments involve local injection of a collagen-digesting enzyme, followed by physical therapy; though this technique avoids a surgical procedure, it is associated with a higher risk of tendon rupture

  • Flexor tenosynovitis

    • Also known as “trigger finger”
    • Caused by thickening or nodularity of the flexor tendon
    • The thickened tendon glides abnormally, causing it to get “caught” at the A1 pulley at the metacarpal head
    • The result is a sticking sensation on flexion or extension that causes extreme pain when force is used to overcome the restriction
    • Unlike flexor tenosynovitis not associated with DM, those associated with DM respond poorly to local steroid injection and often recur, requiring surgical intervention for definitive treatment

  • Diabetic amyotrophy

    • A condition which often causes muscle weakness resulting from a non-inflammatory mononeuropathy
    • Usually affects the thigh, hip, and buttocks
    • EMG can be used to suggest the condition and rule out other conditions

  • Diabetic muscle infarction

    • Typically presents as acute, localized swelling and pain, usually in a lower extremity, that persists for several weeks
    • Creatine kinase (CK) may be elevated or normal
    • Other conditions such as infection and thrombosis must be ruled out
    • MRI can establish the diagnosis, but in the correct clinical setting is not necessary
    • Treatment is conservative, with pain management and activity restriction in the acute phase, followed by physical therapy in later stages

  • Other DM-associated syndromes are discussed elsewhere in this text:

    • DISH
    • Neuropathic arthritis
    • Carpal tunnel syndrome

Thyroid Disease



  • Hyperthyroidism

    • Thyroid acropachy

      • An extreme manifestation of Graves disease
      • Patients exhibit soft tissue swelling of hands, fingers, and toes with clubbing
      • Periostitis is evident on x-ray
      • Often associated with thyroid dermopathy and thyroid ophthal­mopathy

    • Hyperthyroidism is also associated with a non-inflammatory proximal myopathy

      • Usually seen in the setting of toxic multinodular goiter or Graves disease
      • Caused by direct toxic effect of thyroxine on muscle fibers
      • Heat intolerance in addition to muscle fatigue can be a clue to the diagnosis that helps to exclude other diagnoses
      • CK is typically elevated, and thyroid tests suggest hyperthyroidism

  • Hypothyroidism

    • Associated with a myopathy with elevated CK levels and compatible thyroid tests; therefore hypothyroid myopathy must be ruled out when evaluating for inflammatory myopathy
    • Non-inflammatory joint effusions and arthralgias are often seen
    • Can be associated with pseudogout or asymptomatic chondrocalcinosis

Parathyroid Disease: Hyperparathyroidism



  • As with hypothyroidism, hyperparathyroidism can be associated with a non-inflammatory arthritis, as well as pseudogout and chondrocal­cinosis
  • Hyperparathyroidism is also associated with osteopenia and early osteoporosis
  • Osteitis fibrosa cystica is a syndrome in which uncontrolled hyperparathyroidism induces excess bone breakdown, causing the bones to become soft, painful, and deformed

    • May be seen in primary hyperparathyroidism as well as renal osteodystrophy, or hyperparathyroidism associated with chronic kidney disease
    • X-rays show bone cysts and subperiosteal erosions, as well as thin bones and bowing
    • Erosions in the pelvis may mimic sacroiliitis, and must be interpreted in the appropriate clinical setting to arrive at the correct diagnosis

Acromegaly



  • Acromegaly is caused by abnormal increased secretion of growth hormone and its primary mediator, insulin-like growth factor (IGF) 1
  • A wide spectrum of rheumatic diseases is common in acromegaly
  • Manifestations include a non-inflammatory arthritis that can be quite disabling, due to early osteoarthritis and abnormal repair mechanisms
  • Joints affected are primarily the knees, shoulders, and lumbosaral spine
  • X-rays typically show widened joint spaces due to excess cartilage growth early on, followed by more typical findings of osteoarthritis
  • Some patients also develop DISH, associated with the development of diabetes mellitus
  • Premature osteoarthritis with carpal tunnel syndrome are commonly the initial signs of acromegaly
  • Acromegaly can also be associated with pseudogout and chondrocalcinosis
  • Bone changes are usually irreversible even once treatment begins

Infectious Arthritis

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Jul 3, 2016 | Posted by in RHEUMATOLOGY | Comments Off on 9: Miscellaneous arthropathies

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