A 46 yr old female with status epilepticus and chronic kidney disease and anti synthetase syndrome

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I have been given this case to solve in an attempt to understand the topic of " patient clinical data analysis" to develop my competency in reading and comprehending clinical data including history, clinical findings, investigations and come up with diagnosis and treatment plan.

Chief complaint --

A 46 years old female, resident of marrur village, Nalgonda district, came with co

Of seizures associated with haemoptysis since 2days back i.e; Tuesday 03/01/2023 at 5am.


History of presenting illness:

Patient was apparently asymptomatic 2days back, then she had an episode of seizures involving movement of upper and lower limbs, seizures lasted for 3min, associated with haemoptysis and similar episode after 1hour i.e; at 6:00am and 2 similar episodes after admitting in casualty and here the seizures lasted for more than 40 min with loss of consciousness.


Past history: 

Patient was apparently asymptomatic 13 years back. Then she had low back ache and the back pain increased and generalised weakness started for which she went to local hospital and routine investigations are done and found to be having raised creatinine levels, and started on conservative management for her raised creatinine since then.

Since then she is on routine followup with hemogram and serum creatinine levels,and her baseline creatinine levels were 3.2mg/dL. 

In June 2022, she developed fever and cough associated with SOB for which CT chest was done, showing peripheral ground glass opacities and septal thickening was noted.

later after few days, she developed swelling of both lower limbs upto ankles, which were insidious in onset and gradually progressive.

Then she had underwent dialysis for the first time through right IJV line, for 4 hours and was on conservative management since then for 2-3months.

Then 3 months later, she developed fluid filled blebs over the fingers of hand, some of which ruptured on their own and some were pricked by the patient.

she also had eroding and distorted nails and hyperpigmented macules over the face, and itching over the palms, and low grade fever associated with loss of apetite and alopecia, for which she got investigated further, and she was Tested for ANA profile which Positive for Anti Ro 52 and SSA/Ro 60++, and SSB/La+.




Then due to persistent low Hb 5-6g/dL, bone marrow aspiration (from right posterior iliac spine)was done for evaluation of anemia, and found to be having peripheral anemia with cellular marrow and plasma cells are 20%, and euthyroid hyperplasia.

 then she was started on mycophenolate mofetil 360mg, for 3-4months.

In November she developed cough since 1 week,with whitish color sputum, mucoid in consistency and moderate in amount and non blood stained and non foul smelling 

and bilateral swelling of lower limbs till knee,not associated with any redness or trauma,and decreased urine output for 2 days, 

and Shortness of breath MMRC grade 3, and loss of apetite, no abdominal distension, constipation, diarrhoea, facial puffiness, headache and seizure activity at that time,

and she was diagnosed as? Antisynthetase syndrome 

withCLD secondary to autoimmune hepatitis with hypoalbuminemia

along with acute exacerbation of ILD,nand recurrent anemia.

In December,in view of further increase in SOB, and abdominal distension she was taken to second session dialysis, and bronchoalveolar lavage was performed and was found to be having an infective etiology and mucus plugs in the airways. 



On radiograph of chest, she was found to be having left lower lobe consolidation, and PET CT was advised and was done,and they suspected Tuberculosis for which she was on ATT since 20days. 




Current situation: Her seizures continued after admitting on Tuesday morning, each episode is about 2min. CT brain was done in view of recurrent seizures.

Inj lorazepam was given,

Later levitracetam 

and then sodium valproate given as her seizures were not controlled.

Later she had continuous episode of seizures lasting for more than 45 min, then she was sedated with IV MIDAZOLAM and intubated.


General examination:

Patient is in sedation.

On inspection, she has hyperpigmentation on her face and upper limbs












Single Bleb on the right hand

VITALS:

Temperature:afebrile 

BP 160/110mmhg

Pulse 158bpm

RR 37 cpm


SYSTEMIC EXAMINATION

CVS : S1,S2 heard. No murmurs

RS : Bilateral air entry present

Normal vesicular breath sounds were heard

CNS 

As the patient is sedated, I didn't elicit Sensory examination, Motor examination.

Pupils: 

Reflexes:

                        Rt.          Lt 

Biceps:            2+         2+

Triceps           2+.        2+

Supinator.     A.          A

Knee.             A.          A

 Ankle            A.          A


INVESTIGATIONS:

On 03-01-2023:








On 04-01-2023:







Xray 




PROVISIONAL DIAGNOSIS:

STATUS EPILEPTICUS, with CHRONIC KIDNEY DISEASE since13 years with AUTOIMMUNE INVOLVEMENT

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