Adult-Onset Still’s Disease

Epidemiology

  • History: “Still’s disease” was first described in children in 1896 by the English physician, Sir George Frederic Still -> this entity should probably now be termed “Systemic Onset Juvenile Idiopathic Arthritis”
  • Bimodal Age Distribution: however, cases have been reported in patients >70 y/o
    • Peak Between Age 15-25
    • Peak Between Age 36-46

Etiology

  • Unknown
    • While viral pathogens (Rubella, Parvovirus B19), Mycoplasma Pneumoniae, and Yersinia Enterocolitica have been suspected to be etiologic, no definitive role for these pathogens has been proven

Diagnosis

  • Anti-Nuclear Antibody (ANA): negative
    • Low titer positivity occurs in 10% of cases
  • Rheumatoid Factor (RF): negative
    • Low titer positivity occurs in 10% of cases

Clinical-Yamaguchi Criteria

General Comments

  • Sensitivity of Criteria: Yamaguchi criteria have the highest sensitivity of all of the published criteria
  • Diagnostic Requirements: one of the following
    • 2 Major Criteria
    • 3 Minor Criteria

Major Criteria

  • Fever: at least 39 degrees C and lasting at least 1 week
  • Arthritis/Arthralgias: lasting at least 2 wks
  • Salmon-Colored Non-Pruritic Macular/Maculopapular Rash: usually found over trunk or extremities during febrile episodes
  • Leukocytosis (see Leukocytosis, [[Leukocytosis]]): at least 10k with at least 80% granulocytes

Minor Criteria

  • Pharyngitis
  • Lymphadenopathy
  • Hepatomegaly or Splenomegaly
  • Abnormal Liver Function Tests (LFT’s)
    • Elevated AST
    • Elevated ALT
    • Elevated LDH
  • Negative ANA and RF

Clinical Manifestations

General Comments

  • Must exclude conditions which might mimic adult-onset Still’s disease

Cardiac Manifestations

Dermatologic Manifestations

  • Evanescent Salmon-Colored Non-Pruritic Macular/Maculopapular Rash (see xxxx, [[xxx]])
    • Diagnosis: pathology is non-specific but demonstrates dermal edema and mild perivascular inflammation (lymphocytes, histiocytes) in superficial dermis
      • Immunofluorescence: slight deposition of C3 in vessel walls
      • Skin biopsy is important to rule out Sweet’s syndrome, vasculitis, and other disorders
    • Clinical
      • Predominantly involves trunk or extremities (but may involve face, palms of hands, soles of feet, or areas of tight clothing, such as under beltline or beneath the breasts)
      • Typically present during febrile episodes
      • Koebner Phenomenon: eruption can be invoked by stroking of the skin

Gastroenterologic Manifestations

  • Abnormal Liver Function Tests (LFT’s)
    • Elevated AST
    • Elevated ALT
    • Elevated LDH
  • Fulminant Hepatic Failure (see Fulminant Hepatic Failure, [[Fulminant Hepatic Failure]]): cases have been reported
  • Hepatomegaly (see Hepatomegaly, [[Hepatomegaly]])

Hematologic Manifestations

  • Disseminated Intravascular Coagulation (DIC) (see Disseminated Intravascular Coagulation, [[Disseminated Intravascular Coagulation]])
  • Hyperferritinemia (see Hyperferritinemia, [[Hyperferritinemia]])
    • Decreased Percentage of Glycosylated Ferritin: percentage of ferritin which is glycosylated is lower than that in other rheumatologic diseases -> reported to be between 3.7-30%
      • Best to use in combination with serum ferritin
  • Leukocytosis (see Leukocytosis, [[Leukocytosis]]): at least 10k with at least 80% granulocytes
  • Lymphadenopathy (see Lymphadenopathy, [[Lymphadenopathy]])
  • Microangiopathic Hemolytic Anemia (MAHA) (see Hemolytic Anemia, [[Hemolytic Anemia]])
  • Pure Red Cell Aplasia (see xxxx, [[]])
  • Reactive Hemophagocytic Syndrome (Macrophage Activation Syndrome) (see Hemophagocytic Syndrome, [[Hemophagocytic Syndrome]])
    • Diagnosis
      • Bone Marrow Biopsy: xxx
  • Splenomegaly (see Splenomegaly, [[Splenomegaly]])
  • Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic Syndrome (see xxxx, [[Thrombotic Thrombocytopenic Purpura]])

Pulmonary Manifestations

Rheumatologic Manifestations

  • Arthralgias (see Arthralgias, [[Arthralgias]])
  • Arthritis (see Arthritis, [[Arthritis]])
    • May evolve over months into a destructive polyarthritis
    • Fusion of wrist joints is characteristic (but seen in only a minority of cases): non-erosive narrowing of carpometacarpal and intercarpal joint spaces of the wrists is a classic finding
  • Myalgias (see Myalgias, [[Myalgias]])

Other Manifestations

  • Quotidian (Daily) or Double-Quotidian (Twice Daily) Fevers (see Fever, [[Fever]])
    • Temperature fluctuation may be dramatic: changes of 4 degrees can occur within 4 hrs
    • Fever may persist between spikes in 20-30% of cases

Clinical-Disease Patterns

  • Monophasic Pattern (33% of cases): course lasts only weeks-months, completely resolving in <1 year
    • Predominant Clinical Findings: fever, rash, serositis, hepatosplenomegaly
  • Intermittent Pattern (33% of cases): one or more disease flares with/without articular symptoms (remissions between episodes last weeks-1-2 years)
  • Chronic Pattern (33% of cases): persistently active disease with predominant articular symptoms (and potentially destructive arthritis)

Treatment

Corticosteroids

Anti-TNF Therapy

Other


References

  • Evaluation of serum ferritin as a marker for adult Still’s disease activity. Ann Rheum Dis. May 1992; 51(5): 683–685 [MEDLINE]
  • Severe Systemic Inflammatory Response Syndrome With Shock and ARDS Resulting From Still’s Disease. Clinical Response With High-Dose Pulse Methylprednisolone Therapy. (CHEST 1999; 115:1738–1740)