4 Dec 2020

8/13/20 SLE and Lupus Lymphadenitis

PGY3 Edward Lin presented a case of a 46 y/o F with no PMH presenting with 3 weeks of edema, joint pain, rash and sore throat. 4 weeks prior to presentation, patient had a deep tissue massage that resulted with diffuse muscle soreness. 3 weeks prior, she developed bilateral knee pain that was worse with ambulation, improved with rest, non-radiating, associated with diffuse myalgia. She was trialed on naproxen which initially helped with the pain but she started developing b/l LE and b/l hand swelling followed by a non-pruritic rash on her bilateral lower and upper extremity 3 ays later. 1 week prior, she developed a sore throat with discovery of a mass on her hard palate. The next day, she developed nausea and subjective fevers accompanied by diffuse tremors, redness of the face and feet and weakness of the hands. No significant PMH, FH or home medications. Denies tobacco/ethanol/drug use. Vitals significant for temp of 39C, HR 92, RR 15, BP 127/78, satting 99% on RA. Exam significant for erythema with petechiae on soft palate, 2cmx2cm white mass with erythematous borders on the hard palate, +anterior L cervical LAD, 3/6 systolic murmur heard through al 4 valve regions, tenderness in R hand 3rd PIP, b/l hand swelling, b/l LE non-pitting edema and erythematous, blanching rash on the b/l LE that is flat and multiple erythematous spots on b/l cheeks not sparing nasal labial folds.

CBC: WBC 3.0 > Hb 13.3/Hct 38.7 <Plt 315
CMP:
Na 134/K 5.1/Cl 98/HCO3 24/BUN 18/Cr 0.76 <Glucose 98
ALP 50>T.protein 8.2/Albumin 3.9/AST 113/ALT 116/Tbili 0.5/DBili <0.2

Coags: PT 12.6, INR 0.96

Infectious workup:

  • Lactate 1.4 (normal 0.5-2.2)
  • Procal 0.17 ng/mL
  • BCx negative x2
  • Fungal Cx negative
  • HIV negative
  • RPR negative
  • Acute hepatitis panel negative
  • EBV IgG positive only, CMV IgG positive only, HSV-6 not detected, VZV negative
  • Parvovirus negative
  • Rickettsia typhi negative
  • UA 100 protein, negative leuks/nitrites

Heme workup:

  • Iron panel: Iron 45, IBC and Iron sat unable to report due to ferritin interference, Ferritin 2336
  • LDH 345 (normal 90-220)
  • Haptoglobin 365
  • SPEP: immunofixation reveals no monoclonal protein bands
  • UPEP: high concentration of polyclonal gammglobulins. Faint banding is seen in IgG, K and L consistent with oligoclonal pattern
  • Kappa LC free 94.3 (normal 3.3-19.4)
  • Lambda LC free 96.7 (normal 5.7-26.3)
  • K/L ratio 0.98 (normal 0.26-1.65)
  • APLS labs initially positive but was negative on repeat several weeks later
  • IL 2R 5151 pg/mL (normal 532-1891)

Rheum workup:

  • ESR 75 (normal 0-29)
  • CRP 3.8 (normal <7)
  • ANA positive, homogeneous, 1:1280
  • dsDNA positive, 1:320
  • ANCA negative
  • C3 36 (normal 90-180)
  • C4 4.6 (normal 10-40)
  • SM/RNP Ab >8 positive
  • RF 14 (normal <13)
  • Anti-CCP <8 (normal <16)
  • Anti-centromere negative
  • Anti-Jo-1 negative
  • Anti-mitochondrial negative
  • Anti-LMK negative
  • Scl-70 Ab negative
  • Anti-SSA >8 positive, Anti-SSB negative
  • CK 29 U/L
  • Aldolase 5.7 U/L (normal <8.1)

CT Soft tissue neck w/ contrast revealed R supraclavicular, subpectoral and axillary LAD.

CT C/A/P showed again the round, enhancing, asymmetrically enlarge LAD in R axillary, subpectoral and subclavicular stations. Also noted a heterogenous L adnexal lesion measuring 2.6cm x 3.4cm.
EL4

PET/CT showed several pathologically enlarged and FDG avid L level 5 cervical and R >L axillary lymph nodes. No significant metabolic activity of the L adnexal lesion which is inseparable from the uterus.

FNA of R supraclavicular LN showed nonspecific findings of polymorphous lymphoid population with extensive necrosis.

Skin biopsy showed acute SLE vs dermatomyositis vs other connective tissue disease process.

Excisional LN biopsy of the L level 5 LN revealed lupus lymphadenitis.

Treatment: Patient was started on steroids after extensive workup.

Take home points:

  1. LAD in SLE raises the differential diagnosis of lupus lymphadenitis vs Kikuchi-Fujimoto Disease vs malignancy (lymphoma vs metastasis) vs infection.
  2. Clinically and histologically, lupus lymphadenitis and Kikuchi Fujimoto Disease can be closely similar.
    1. Lupus lymphadenitis can have paracortical necrosis with inflammatory cells. It would often have haemotoxylin bodies (aggregates of degenerated nuclear debris), abundant plasma cells and capsular or pericapsular inflammation.
    2. Kikuchi-Fujimoto Disease (as known as histiocytic necrotizing lymphadenitis) will show CD68 positive plasmacytoid monocytes and histiocytes with predominantly CD8-positive T lymphocytes. There is no treatment for Kikuchi Fujimoto disease. For patients with severe disease, mediations for SLE, specifically hydroxychloroquine and glucocorticoids have been used successfully to treat the disease.

Sentinel Article:
Kikuchi-Fujimoto Disease

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