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SLE (SYSTEMIC LUPUS ERYTHEMATOSUS)

Introduction

Systemic lupus erythematosus (SLE) is a multisystemic, autoimmune, rheumatic disease involving the joints and skin in up to 90% of affected individuals. SLE affects various organ systems simultaneously, and its course is typically recurrent, with periods of relative remission followed by relapses. Hand involvement is a common manifestation and can vary considerably from mild arthralgia to severely deformed hands. Treatment varies widely depending on the systemic involvement and disease severity, but conservative management generally focuses on a course of disease-modifying antirheumatic drugs (DMARDs) and other pharmacologic interventions, while surgical procedures like tendon rebalancing, arthrodesis and arthroplasty are typically reserved for patients with advanced deformity and severe functional impairments.1,2

Pathophysiology

  • Genetic, epigenetic, ethnic, immunoregulatory, hormonal, and environmental factors are all believed to be associated with its development
  • Many innate and acquired immune disturbances occur in SLE and are therefore proposed mechanisms of its pathophysiology
    • T- and B-cell hyperactivation, increased apoptosis, defective immune complex clearance, high ratio of CD4:CD8 T-cells, and impaired autoimmune tolerance3
  • SLE arthritis is traditionally considered to be mild, reversible, and non-erosive
    • 5–15% of cases progress to deforming arthropathy, which is either erosive as in rhupus syndrome or non-erosive as in Jaccoud’s arthropathy5
      • Jaccoud’s arthropathy is characterized by subluxation of metacarpophalangeal (MP) joints, ulnar deviation, swan neck deformity, and thumb deformity due to changes in soft tissues
    • Joint deformities in the hand occur as a result of ligament and volar plate laxity, as well as extensor tendon subluxation, resulting in an imbalance of forces acting on the joints. The deformity is actively correctible initially but may progress to a fixed deformity.
      • The characteristic deformity at the MCP joint is flexion, with ulnar deviation and volar subluxation resulting from extensor tendon subluxation ulnar to the metacarpal head. 
        • Progressive volar shift of the extensor tendon causes the proximal phalanx to subluxate volarly. 
        • Intrinsic muscle shortening, especially of the ulnar muscles, occurs as a result of the volar subluxation of the proximal phalanx and MCP joint ulnar deviation.
        • The resultant intrinsic tightness causes hyperextension deformity at the proximal interphalangeal joint.
          • Clinically, the patient progresses from being able to extend the affected MCP joints actively to loss of active extension but maintenance of passive extension.
          • In later stages, the deformity may become fixed with an inability to extend the MCP joints even passively.

Related Anatomy

  • MP joints
  • Interphalangeal (IP) joints
  • Hand flexor tendon
  • Wrist joint
  • Wrist flexor tendon
  • Wrist extensor tendon

Incidence 

  • SLE typically presents between ages 15-44 years and has a prevalence of ~5.8-130 per 100,0006, with ≥1.5 million Americans currently affected7
  • SLE is 2-3 times more prevalent in African-Americans than in Caucasians, and it is 10 times more common in females than in males8
  • Inflammatory musculoskeletal involvement is one of the most common manifestations of SLE, affecting up to 95% of patients9
  • Most important predictors of disease progression are older age at diagnosis, African-American race, and low income4
  • Possible risk factors include cigarette smoking, administration of estrogen to postmenopausal women, exposure to UV light, diabetes, and being overweight3

Related Conditions

  • Rheumatoid arthritis
  • Rhupus syndrome
  • Jaccoud’s arthropathy
  • Leukemia
  • Leukopenia
  • Anemia
  • Osteoarthritis
  • Kidney disease
  • Pericarditis
  • Myocarditis
  • Atherosclerosis
  • Pleuritis
  • Pancreatitis
  • Central nervous system disease
  • Osteonecrosis
  • Vision problems
  • Raynaud’s phenomenon
  • Vasculitis
  • Osteoporosis
  • Antiphospholipid syndrome
  • Ophthalmologic involvement
  • Carpal tunnel syndrome
  • Trigger finger
  • The most frequently affected joints are the MP, IP, wrist, and knee joints9

Differential Diagnosis

  • Adult-onset Still disease
  • B-cell lymphoma
  • Behçet syndrome
  • Chronic fatigue syndrome
  • Dermatomyositis
  • Endocarditis
  • Epstein-Barr virus
  • Fibromyalgia
  • Hepatitis C
  • HIV infection
  • Inflammatory bowel disease
  • Kikuchi's disease
  • Lyme disease
  • Mixed connective-tissue disease
  • Multiple sclerosis
  • Polymyositis
  • Psoriatic arthritis
  • Reactive arthritis
  • Rheumatoid arthritis
  • Rhupus
  • Sarcoidosis
  • Scleroderma
  • Serum sickness
  • Sjögren’s syndrome
  • Systemic sclerosis
  • Thrombotic thrombocytopenia purpura
  • Thyroid disease
  • Undifferentiated connective-tissue disease
  • Vasculitis

Special Laboratory Work-up Diagnosis

  • The initial evaluation should include:
    • Complete blood count (CBC)
    • Comprehensive metabolic panel (CMP)
    • Urinalysis with microscopy (UA)
    • Antiphospholipid antibodies
    • Acute phase reactants ie. Estimated sedimentation rate (ESR) and C-reactive protein (CRP)
    • Serum creatinine and estimated glomerular filtration rate (eGFR)
    • Antinuclear antibody (ANA) test4 -- if this is positive, additional tests to be ordered:
      • C3 and C4 complement or CH50 level tests
      • Anti-dsDNA antibodies
      • Anti-sm antibodies
      • Anti-Ro/SSA and Anti-La/SSB
      • Anti-U1RNP antibody
      • Antiribosomal P protein antibody
ICD-10 Codes

  • SLE (SYSTEMIC LUPUS ERYTHEMATOSUS)

    Diagnostic Guide Name

    SLE (SYSTEMIC LUPUS ERYTHEMATOSUS)

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    SLE (SYSTEMIC LUPUS ERYTHEMATOSUS)M32.9   

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
SLE Hand Deformities
  • Bilateral SLE hand deformities. Note the ulnar deviation of the fingers and the MP joint swelling and deformity especially on the right.
    Bilateral SLE hand deformities. Note the ulnar deviation of the fingers and the MP joint swelling and deformity especially on the right.
Symptoms
Pain often associated with hand deformity, numbness, joint swelling and stiffness and loss of strength
Synovitis, avascular necrosis and Jaccoud's arthropathy
Myalgia, tenosynovitis, tendon derangement, tearing and functional impairments
Photosensitivity
Pleuritic chest pain and shortness of breath
Fatigue and sensitivity to cold
Headaches, confusion, and memory loss
Mouth sores
Fever
Hair loss
Recurrent miscarriages
A butterfly facial (malar) rash
Typical History

The typical patient is a 42-year-old African-American woman who presents with a range of symptoms affecting several systems, including a malar rash, chest pain, shortness of breath, and fatigue, as well as arthralgia, stiffness, swelling, and loss of strength in her hands and wrists. The rheumatoid arthritis-like symptoms have advanced, which now interferes with her ability to perform many basic daily activities requiring the use of her hands.

Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
SLE X-ray Deformities
  • SLE X-ray (AP) with severe MP joint subluxations and dislocations without joint erosions. Note the boutonniere deformity in index finger.
    SLE X-ray (AP) with severe MP joint subluxations and dislocations without joint erosions. Note the boutonniere deformity in index finger.
Treatment Options
Conservative

Nonpharmacologic and Preventative

  • Sun protection
  • Diet and nutrition
  • Exercise
  • Smoking cessation
  • Immunizations
  • Treating comorbidities

Conservative

  • All patients should receive education, counseling, and support, and the general treatment approach should be similar to that for rheumatoid arthritis4
  • Intermittent flares are usually treated with NSAIDs and salicylates
  • For refractory arthritis immunosuppressive therapy may be used
  • Disease modifying anti-rheumatic drugs (DMARDs)
    • Hydroxychloroquine or Chloroquine - first line of treatment for any degree of SLE
    • Methotrexate
    • Azathioprine and mycophenolate
    • Rituximab
    • Mycophenolate
    • Cyclophosphamide
    • Immune globulin IV
    • Adjunctive therapy [to treat joint pain4]
      • Nonsteroidal anti-inflammatory drugs (NSAIDs)
      • Low-dose systemic glucocorticoids (eg, methylprednisolone, prednisone)
      • Belimumab - usually added if resistant first line treatment medications
      • Cyclosporine
  • Physical therapy, including aerobic exercise and passive modalities
Operative
  • May be necessary for patients presenting with advanced joint disease leading to hand and/or wrist deformities and significant functional limitations
  • Comorbidities from disease of other organ systems must be evaluated and managed preoperatively.
  • Extensor tendons at the MCP joints dislocate in an ulnar direction, eventually residing volar to the joint axis.
    • If joint can be reduced passively, the extensor tendons should be tenodesed to the proximal phalanx dorsally and realignment of the sagittal bands.
    • If the joint is fixed, a metacarpal head resection arthroplasty with silicone implant is indicated.
      • Release of tight ulnar intrinsics, volar plates and volar portion of collateral ligaments may not be sufficient to reduce the fixed deformity.
  • Swan neck deformity caused by laxity and tendon imbalance at the PIP joint
    • Early PIP hyperextension can be treated with figure-of-eight orthoses.
    • At an intermediate stage, a flexor digitorum superficialis tenodesis can correct the deformity.
    • With a fixed swan neck, arthrodesis of the joint may be indicated.
  • Thumb collapse and instability often occur with disease progression.
    • An arthrodesis may be required to stabilize the MCP and IP joints
  • Carpal deformities include carpal collapse, ulnar carpal translocation, and volar lunate dislocation with dorsal subluxation of the ulnar head -- common but may not be symptomatic -- severe ulnar head prominence may result in secondary extensor tendon ruptures.
    • Limited or total wrist arthrodesis +/- distal ulnar resection and tendon stabilization can be helpful.
Complications
  • Infection
  • Cardiovascular abnormalities
  • Retinal toxicity
  • Nephritis
  • Renal abnormalities
  • Hemolytic anemia
  • Thrombocytopenia
  • Tendon Ruptures
  • Extensor Tendon Subluxation
  • Glucocorticoid-induced avascular necrosis and osteoporosis
Outcomes
  • Mortality from SLE has improved dramatically over the last 50 years, from a 5-year survival rate of 50% in the 1950s to 95% in 20173
Key Educational Points
  • SLE is difficult to diagnose because many of its symptoms are nonspecific and overlap with those of more common conditions; biomarkers are often negative or normal early in the course of the illness4
  • As opposed to rheumatoid arthritis, articular cartilage of the small joints of the hand is typically normal in SLE.
  • Arthritis and arthralgias have been noted in up to 95% of patients with SLE and can precede the diagnosis of SLE by months or years
  • The arthritis tends to be migratory, compared with RA and plain radiographic evidence of erosions is rare. Morning stiffness is usually brief, and pain may exceed objective physical findings.
  • Because patients with SLE are living longer, the focus of care should be comprehensive and include preventive services in addition to specific treatment for the hand deformities.4
  • Joint arthrodesis or arthroplasty should be considered over soft tissue reconstruction in cases of painful intercarpal collapse with volar subluxation10
  • If MP arthroplasties are performed, early intervention when the joints can still be passively corrected is recommended for better results10
  • Patient shold be evaluated frequently because the disease may progress and result in fixed deformities.
References

Cited

  1. Johnsson P, Sandqvist G, Nilsson JÅ, et al. Hand function and performance of daily activities in systemic lupus erythematosus: a clinical study. Lupus 2015;24(8):827-34. PMID: 25542902
  2. Lins CF, Lima de Sá Ribeiro D, Dourado Santos WG, et al. Ultrasound Findings on Hands and Wrists of Patients with Systemic Lupus Erythematosus: Relationship with Physical Examination. Ultrasound Med Biol 2017;43(9):1764-8. PMID: 28602490
  3. Bakshi J, Segura BT, Wincup C, Rahman A. Unmet Needs in the Pathogenesis and Treatment of Systemic Lupus Erythematosus. Clin Rev Allergy Immunol 2017 Epub ahead of print. PMID: 28853005
  4. Lam NC, Ghetu MV, Bieniek ML. Systemic Lupus Erythematosus: Primary Care Approach to Diagnosis and Management. Am Fam Physician 2016;94(4):284-94. PMID: 27548593
  5. Ruano CA, Malheiro R, Oliveira JF, et al. Ultrasound detects subclinical joint inflammation in the hands and wrists of patients with systemic lupus erythematosus without musculoskeletal symptoms. Lupus Sci Med 2017;4(1):e000184. PMID: 28123769
  6. Jarukitsopa S, Hoganson DD, Crowson CS, et al. Epidemiology of systemic lupus erythematosus and cutaneous lupus erythematosus in a predominantly white population in the United States. Arthritis Care Res (Hoboken) 2015;67(6):817-28. PMID: 25369985
  7. “What is lupus?” Lupus Foundation of America. Published 2015. Retrieved 20 Sept 2017 from https://resources.lupus.org/entry/what-is-lupus.
  8. Somers EC, Marder W, Cagnoli P, et al. Population-based incidence and prevalence of systemic lupus erythematosus: the Michigan Lupus Epidemiology and Surveillance program. Arthritis Rheumatol 2014;66(2):369-78. PMID: 24504809
  9. Gabba A, Piga M, Vacca A, et al. Joint and tendon involvement in systemic lupus erythematosus: an ultrasound study of hands and wrists in 108 patients. Rheumatology (Oxford) 2012;51(12):2278-85. PMID: 22956550
  10. Dray GJ, Millender LH, Nalebuff EA, Philips C. The surgical treatment of hand deformities in systemic lupus erythematosis. J Hand Surg Am 1981;6(4):339-45. PMID: 7252104

New Articles

  1. Lins CF, Lima de Sá Ribeiro D, Dourado Santos WG, et al. Ultrasound Findings on Hands and Wrists of Patients with Systemic Lupus Erythematosus: Relationship with Physical Examination. Ultrasound Med Biol 2017;43(9):1764-8. PMID: 28602490
  2. Ruano CA, Malheiro R, Oliveira JF, et al. Ultrasound detects subclinical joint inflammation in the hands and wrists of patients with systemic lupus erythematosus without musculoskeletal symptoms. Lupus Sci Med 2017;4(1):e000184. PMID: 28123769

Reviews

  1. Bakshi J, Segura BT, Wincup C, Rahman A. Unmet Needs in the Pathogenesis and Treatment of Systemic Lupus Erythematosus. Clin Rev Allergy Immunol 2017. PMID: 28853005
  2. Lam NC, Ghetu MV, Bieniek ML. Systemic Lupus Erythematosus: Primary Care Approach to Diagnosis and Management. Am Fam Physician 2016;94(4):284-94. PMID: 27548593

Classics

  1. Grosse AB. Lupus Erythematosus. Cal State J Med 1903;1(11):322-3. PMID: 18732921
  2. Thin G. On the Pathology of Lupus Erythematosus. Med Chir Trans 1875;58:59-66.1. PMID: 20896450
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