26 May 2022

8/10/20 Adult Onset Still’s Disease

At Goldstein Report, PGY3 Hannah Obasi presented a case of a 40 y/o F with recently diagnosed Type II DM (HbA1c 10.3%) presents with rash, fever and arthralgias. Patient was in her usual state of health until 2 weeks prior to admission when she developed rash on bilateral shins after eating Chinese food. The rash was pruritic and erythematous, initially improved but worsened, appearing over her abdomen, chest and back. 1 week prior to admission, the patient developed daily fevers and started having swelling and pain of her bilateral knees, wrists, elbows, shoulders and ankle along with a sore throat, dry cough and diarrhea. Only home medication was metformin. No family history of autoimmune disorder. She was born in Mexico, no recent travel or sick contact, no history of ETOH/tobacco/drug use. Vitals with temperature of 38.5C, HR 115, RR 18 and BP of 114/75, satting 97% on RA. Exam significant for mild conjunctivae injection bilaterally, no nasal discharge, mild effusion in b/l wrists, knees and ankles, diffusely swollen hands, and pain with ROM on shoulders, wrists, fingers.

Skin Exam:

CBC: WBC 12.1 (78.6% PMNs, 14% leuks)>Hb 13/Hct 38.2<Plt 147
Na 136/K 3.7/Cl 98/HCO3 24/BUN 7/Cr 0.46 <Glucose 285
ALP 94>T.protein 6.3/Albumin 3.8/AST 187/ALT 214/Tbili 0.4/Dbili <0.2

Infectious workup:

  • BCx negative x2
  • UA: trace leuks (4-10/HPF), negative leuks, moderate blood (RBC 0-2 per HPF)
  • UCx negative
  • HIV negative, RPR negative
  • Acute hepatitis panel negative
  • Flu/RSV negative
  • Rapid Group A Ag negative, Anti-ASO <50 IU/mL
  • Brucella IgM/IgG negative
  • HSV 1/2 not detected
  • EBV: hetero mono negative, Ab IgM 29.2 U/ml (H), IgG <36 U/mL
  • CMV Ab IgM <30, IgG 4.8 U/mL (H)
  • Cocci Ab <1:2
  • Rubella IgM <20 AU/mL
  • Ricketssia: both R typhi IgM/IgG and RMSF IgM/IgG are not dectected
  • Parvovirus IgM 0.3, IgG 8 (H)
  • Salmonella/Shigella stool culture not detected

Hematology workup:

  • Lab Peripheral smear: normal platelet morphology, ovalocytes present, vaculolated neutrophils present, Dohl bodies present, Toxic granulation present
  • Ferritin 6699
  • LDH 584 U/L (normal 90-220)

Rheum workup:

  • ESR 66 (normal <29)
  • CRP 128.2 (normal <7)
  • ANA 1:80 homogenous
  • dsDNA negative
  • C3 205 (normal l90-180)
  • C4 37.6 (normal 10-40)
  • ANCA negative
  • CK 48 U/L
  • RF 14 (normal <13)
  • CCP <8 (normal <16)

Wrist and Knee XR with no evidence of acute osseous injury or joint disease:

Abdominal US with hepatomegaly (liver measuring 18.6cm at R midclavicular line) but otherwise unremarkable US.

Treatment: After infection was ruled out, patient was started on Methylprednisolone 12mg Q8H for concern of Adult Onset Still’s Disease with resolution of fevers, arthralgias and rash. She was transitioned to Prednisone 50mg qday upon discharge.

Take home points:

  1. Adult Onset Still’s Disease (AoSD) classic triad: Salmon colored rash, persistent high spiking fevers, arthralgias/arthritis. It is a diagnosis of exclusion.
  2. Yamaguchi criteria: Need 5+ with at least 2 major criteria satisfied for diagnosis
    1. Major criteria: Fever of at least 39C lasting at least 1 week, arthralgia/arthritis lasting 2 weeks or longer, characteristic skin rash (nonpruritic macular or maculopapular salmon-colored over trunk or extremities during febrile episodes), WBC 10K + with at least 80% PMNs
    2. Minor criteria: sore throat, lymphadenopathy, hepatomegaly/splenomegaly, elevation in liver enzymes concentrations, RF and ANA negative
  3. Treatment
    1. Mild: fever + rash + arthralgia + mild arthritis: NSADs or low dose glucocorticoid therapy
    2. Moderate: high fevers + debilitating joint symptoms + internal organ involvement: glucocorticoids (0.5 – 1 mg/kg/day) or non-biologic/biologic DMARDs
    3. Severe: life threatening organ involvement (e.g severe hepatic involvement, DIC): high dose or pulse glucocorticoids, biologic (IL-1 or IL-6 inhibitors)

Sentinel Articles:
Adult Onset Still’s Disease
A multicenter study of patients with adult-onset Still’s disease compared with systemic juvenile idiopathic arthritis


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