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A Dance to the Autoimmune Tune !!

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Hello everyone i am dr prajj navi karki junior resident in the department of clinical immunology and rheumatology at st john’s medical college hospital bangalore today i’ll be narrating an interesting short a dance to the autoimmune tune this 25 year old male previously diagnosed with systemic lupus erythematosus and class 4 lupus nephritis presented to ropd

With involuntary movements of the right side of his body these movements were subacute in onset brief spontaneous and involuntary that primarily involved the distal parts of his right upper limb classically termed as the milkmaid’s grip it also involved similar movements in his right foot and twitching of his right-sided facial muscles however there was no history

Suggestive of prior focal neurological deficits or cranial neuropathy and minimal scale examination was normal his baseline investigations include a normal routine investigation panel a strongly positive anti-nuclear antibody a positive anti-double-stranded dna and a positive apply workup with marginally elevated anti-cardiolypin antibody igg additionally complements

Were decreased and kidney biopsy showed class 4 lupus nephritis with misangel and peripheral deposits of igg and c1q after considering the various differentials of korea an mri of the brain and an mr venogram were performed that were normal additionally c3 c4 and other routine investigations were also within normal limits so based on the clinical history physical

Examination histopath and radiological investigations a diagnosis of systemic lupus erythematosus and class iv lupus nephritis with immune-mediated hemichoria was established prior to the onset of korea he was on treatment with steroids mycophenolate morphetal and hydroxychloroquine the steroids were escalated and mycophenolate morphetal was optimized as the

Symptoms interfered with his daily activities and work a low dose of aspirin and trihexy phenol were also added in this presentation we focus on why korea occurs in lupus korea is an uncommon manifestation of lupus and its pathology still remains unclear two hypotheses have been proposed the first is the presence of antiphospholipid antibodies and the occurrence

Of chorea in lupus antiphospholipid antibodies cross react with other phospholipids that lead to direct antibody mediated damage to the phospholipid containing areas of the basal ganglia the second is the vascular hypothesis that is the occurrence of a few thrombotic events that lead to reversible ischemia in the basal ganglia microvascular a few take home points

Include neuropsychiatric sle occurs in 10 to 60 percent of lupus in the course of illness among which movement disorders account to less than five percent and korea one to two percent and even nine percent in childhood making it a very rare manifestation of lupus although korea usually occurs during the course of sle it may also be the presenting manifestation of

The illness in one of the largest case series including 32 patients with korea 11 had korea as the presenting manifestation of lupus the occurrence of korea and lupus has found to be associated with the presence of antiphospholipid antibodies a number of studies indicate immunoglobulin igg anticardiolipin antibody as the main antibody related to lupus chorea as we

Can see in our patient to conclude lupus rarely manifests with movement disorders like choreoattitosis and antiphospholipid antibodies are often found in such patients therefore while considering the various differentials of korea sle must not be overlooked thank you you

Transcribed from video
A Dance to the Autoimmune Tune !! By Indian Rheumatology Association