0116 - "EXTRA-CRITERION" MANIFESTATIONS DUE TO NEPHROPATHY DUE TO APS: CASE

REPORT
Authors: Rivadeneira CS¹, Guevara-pacheco DG¹, Lamos-duarte AF¹, Buitrago JF¹, Gallego-cardona L¹, Cajamarca-baron JH², Cubides HH², Arredondo AM², Escobar A².
1.Rheumatology Fellow - Fundación Universitaria Ciencias de la Salud –Hospital de San José, Bogotá
2.Rheumatology instructor- Fundación Universitaria Ciencias de la Salud - Hospital de San José, Bogotá

Correspondence contact: Carlos Rivadeneira, csrivadeneira@fucsalud.edu.co

Image 1. Brain magnetic resonance

Purpose or objective: Antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilia
characterized by recurrent vascular thrombosis and/or pregnancy morbidity, in the presence of persistent
antiphospholipid (aPL) titers.

Clinical phenotypes outside the Sapporo classification criteria have been evaluated, clinical profiles:

1. Obstetric APS
2. Thrombotic APS
3. Microvascular APS
4. Catastrophic APS (CAPS)
5. Nonthrombotic APS: thrombocytopenia, skin, heart valve disease and nephropathy

Patients and methods: Case report and literature review a) FLAIR sequence axial section; b) Multiple left frontoparietal hyperintensities (LMCA) with white matter
involvement; c) Hyperintensity in the caudate and lenticular nuclei.

Table 1. Laboratory tests

Results: A 42-year-old man, with high blood pressure, chronic kidney disease and myocardial infarction, was
admitted to the emergency room due to a 3-day history of dysarthria, motor aphasia, dysgraphia associated with
right hemiparesis and syncopal episodes; normotensive physical examination, no arrhythmias, no skin lesions,
neurological examination with mental examination with motor aphasia, agraphia, alexia, strength and reflexes 4
limbs normal; brain magnetic resonance observed a acute ischemic event (Image 1), with extension studies with
thrombocytopenia, positive aPL , and proteinuria (Table 1); renal biopsy was taken with findings of
membranoproliferative nephropathy and thrombotic microangiopathy.

With the studies obtained, stroke of the left middle cerebral artery (LMCA) was considered as a thrombotic
manifestation of APS with a first triple positive set and "extra-criterion" manifestations due to severe
thrombocytopenia and nephrotic syndrome secondary to APS nephropathy.

Anticoagulation is started, but a decrease in platelets to 1,500 is documented, steroid pulses are indicated,
without response. Subsequently, he presented a new acute event with right hemiparesis, with MRI with stroke in
new territories of the LMCA with hemorrhagic transformation, immunoglobulin was indicated due to
refractoriness to steroids with recovery of platelet count, and initiation of cyclophosphamide due to renal
involvement, presenting adequate medical evolution.
Keywords: Antiphospholipid syndrome; nephropathy, thrombocytopenia
Conclusions: The extra-criterion nontrombotic manifestations of APS include kidney involvement due to aPL nephropathy, it has a low prevalence, it is characterized
as a vascular nephropathy with occlusive lesions of small vessels, related to refractory arterial hypertension, CKD, hematuria, proteinuria associated with positivity of
lupus anticoagulant, hypocomplementemia, with indication of immunomodulation.