27 Nov 2020

11/16/20: Catastrophic anti-phospholipid syndrome (CAPS)

PGY3 Patrick Chang presented a case of a 69 y/o male with history of polysubstance abuse, COPD, chronic pancreatitis, Hepatitis C, Childs A cirrhosis, Type II DM and HTN who was brought in by ambulance for acute encephalopathy.  Per his case worker, the patient was found down in his transitional housing unit, having soiled himself, and unresponsive.  He as last seen well 24 hours prior.  Over the last month, the case worker has noted developing “dementia” and multiple “episodes’ this past month where he has been found down in his own stool.  She states at baseline, the patient is A&Ox4, and behaves and speaks normally outside these “episodes”.  Denies any history of known trauma.  Home medications include ASA 81mg, atorvastatin 80mg, MTP succinate 50mg, lisinopril 5mg, tamsulosin 0.4mg and furosemide 40mg qday.  Social history significant for active cocaine and tobacco use, previous heroin use.

In the ED, patient initially A&Ox2 but by the time on the floor, the patient had become A&Ox0, not following commands.

Vitals: T 36.8, HR 74, RR 19, BP 93/71, O2 sat 99% on RA

Physical Exam significant for cachectic African American male, L gaze preference, normal cardiac and pulm exam, bula present on L thigh and R hand wrist, purpuric lesions, necrotic great R toe, A&Ox0, withdraws to pain.

CBC: WBC 10.7> Hb 10/Hct 31.6 <Plt 21 (MCV 89.3, RDW 14.4%)

CMP:

Na 143/K 4.1/Cl 112/HCO3 18/BUN 105/Cr 4.21 <Glucose 95 (Baseline Cr: 1.2)

ALP 61>T.protein 5.4/Albumin 2.8/AST 393/ALT 77/Tbili 0.7/Dbili 0.4

Ca 8.2, Mg 2.7, Phos 3.3

Coags: PT 19.4, INR 1.65, PTT 44.5

Acute encephalopathy workup initiated, with the following findings:

  • Utox positive for cocaine
  • Ammonia elevated at 69 umol/L (normal: 16-60)
  • Negative ETOH, APAP, ASA levels
  • TSH wnl
  • EEG: abnormal record due to presence of mild to moderate diffuse slowing
  • CTH with no abnormal acute findings
  • MRI brain: extensive nonspecific white matter signal abnormality in the periventricular deep white matter as well as the deep gray nuclei, multiple punctate foci of reduced diffusion involving the R precentral gyrus, R superior frontal gyrus and L superior parietal lobule

Infectious workup initiated, with the following findings:

  • Blood Cx, Fungal Cx, Urine Cx all negative
  • HIV negative, RPR negative
  • Shiga toxin negative
  • Hepatitis panel with reactive Hep C, Hep C VL elevated at 56K
  • TTE with EF of 30-35%, moderate diffuse hypokinesis, no valvular disease noted
  • LP with 4 nucleated cells, 61 glucose, 49 protein
  • CSF bacterial and viral biofire panel negative

Renal workup initiated, with the following findings:

  • UA with >300 protein, >50 RBC and >50 WBC
  • Urine lytes with FeNa 0.5%, FeUrea 29.5%
  • Urine protein/cr: 1.14
  • Cryoglobulin: 6% (marked cryoprecipitate was detected)

Rheum workup done and negative for ANA and ANCA.

Heme workup initiated, with the following findings:

  • Peripheral smear with giant platelets, reactive lymphocytes but no schistocytes noted
  • Complements levels both decreased, CH50 also decreased
  • ADAMSTS13: 51%
  • APLS labs with elevated Cardiolipin IgM and lupus anticoagulant positive
  • Hemolysis labs: Haptoglobin <10, LDH 782 (normal 135-225), Fibrinogen 163 (normal 237-481), DAT negative

Derm performed punch biopsy on R knee: thrombotic vasculopathy.

Treatment: The patient was treated with 3 days of plasmapheresis and 1 day of Rituxan due to concern for catastrophic APLS vs cryoglobulinemia.  He was eventually extubated and improved to A&Ox2.

Teaching points:

  1. Catastrophic antiphospholipid syndrome (CAPS) = exaggerated acute occlusion of small vessels in multiple organs and represents 1% of anti-phospholipid syndrome
    1. Characterized by evidence of involvement of 3+ organs, systems/tissues, development of manifestations simultaneously or in less than a week, confirmation by histopathology of small vessel occlusion and laboratory confirmation of the presence of antiphospholipid antibodies
  2. Mortality rate of 50% and 50% have a trigger within 1 week (trauma, infection, medication, surgery)
  3. Treatment: high dose systemic IV glucocorticoids, plasma exchange, rituximab, anticoagulation
  4. In case series, thre are noted association between CAPS and cryoglobulinemia and significant overlap in presentation.

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