Acute cholecystitis - an isolated unusual presentation of thrombotic microangiopathy of gall bladder due to antiphospholipid syndrome with lupus positivity detected by [۹۹mTc]Tc-mebrofenin scan

Occurrence of small-vessel occlusions (thrombotic microangiopathy, TMA) in association with antiphospholipid antibodies (aPL ab) has been documented. But isolated involvement of gall bladder due to TMA in secondary aPLS (antiphospholipid syndrome) in post-partum status has been unreported. aPL and systemic lupus erythematosus (SLE) are two closely related diseases that not only share lupus susceptibility genes but can also produce thrombosis. Here we present a young lady, recently delivered with SLE experiencing moderate to severe right abdominal pain and occasional vomiting. A hepatobiliary iminodiacetic acid (HIDA) scan revealed non visualization of gall bladder (GB) confirming the diagnosis of acute acalculous cholecystitis (AAC). Investigations revealed high titre of anti-β۲ glycoprotein I (β۲GPI) IgG and anti-Cardiolipin (aCL) IgG antibodies confirming aPL syndrome. Antinuclear antibody, double-strand dsDNA was found to be positive confirming SLE. Open cholecystectomy was performed and histology revealed AAC, vasculitis with small vessel ischemia of gallbladder. GB non visualization on HIDA scan (ACC) due to solitary thrombotic microangiopathy of GB is being highlighted in this case of active SLE and secondary aPLS. Early diagnosis is mandatory to avoid repetitive thrombotic occlusions in active cases of SLE with aPLS.

Keywords:

Thrombotic microangiopathy , Gall bladder , Mebrofenin scan , Acute cholecystitis , Antiphospholipid syndrome