Incomplete Catastrophic Antiphospholipid Syndrome and Heparin-Induced Thrombocytopenia in Diffuse Systemic Sclerosis: A Case Report

Mihaela-Maria Tamșa^(1,*), Ioana Felea⁽¹⁾, Filipescu Ileana^(1.2), Andreea Rapciuc⁽¹⁾, Titus Pop⁽¹⁾, Iulia Popa⁽³⁾, Svetlana Encica⁽³⁾, Simona Rednic^(1,2)

1)Department of Rheumatology, County Emergency Hospital Cluj-Napoca, Romania
2)Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania
3)Nicolae Stăncioiu Heart Institute, Cluj-Napoca, Romania

Background. Catastrophic Antiphospholipid Syndrome (CAPS) and Heparin-Induced Thrombocytopenia (HIT) are rare, life-threatening thrombo-inflammatory conditions that may overlap in patients with systemic autoimmune disease.
Case Report. A 63-year old woman with diffuse cutaneous systemic sclerosis (dcSSc), relapsing polychondritis and established antiphospholipid syndrome developed multiorgan failure after a brief switch from vitamin K antagonist therapy to low molecular weight heparin and a confirmed infection with Clostridium. The hospital course was complicated by suspected HIT, fulminant cardiac dysfunction and widespread microthrombosis despite clean coronary angiography. Autopsy confirmed innumerable myocardial microthrombi, aortic cross mural thrombosis and left subclavian arterial thrombosis, fulfilling three of the four criteria and can be classified as incomplete CAPS.
Conclusions. Early recognition of CAPS and immediate cessation of all heparin products in suspected HIT are critical to improve survival in similar high risk patients.

Keywords: incomplete catastrophic antiphospholipid syndrome; heparin-induced thrombocytopenia; systemic sclerosis; microvascular thrombosis

Corresponding author(s): Mihaela Maria Tamșa, Department of Rheumatology, County Emergency Hospital Cluj-Napoca, Romania; e-mail: tamsa.mihaela@gmail.com