sle rta.pdf

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SLE - IT'S ATYPICAL CLINICAL PRESENTATION G.Ravi Kiran, Dr.V. Chandrashekar, Dr. Bikshapathi Rao Department of General Medicine, MGM Hospital Kakatiya Medical College, Warangal, Telangana Introduction Hypokalemic periodic paralysis (HPP) is a form of metabolic myopathy, characterized by hypokalemia, acute flaccid paralysis, and potentially fatal episodes of muscle weakness through the involvement of the respiratory muscles, and cardiac arrhythmias. It can be a primary disorder or secondary with causes like renal tubular acidosis (RTA), Distal RTA (dRTA) is the most common form & it's association with autoimmune diseases is well documented Case Presentation A 34 year old female patient presented with C/C Low grade fever & weight loss - 5 months, Weakness of both lower limbs - 15 days , Inability to walk - 6 days, Past History: Patient had history of similar weakness of lower limbs 1 episode - 45 days back, She is Not a Known Case of HTN, DM, Asthma, Epilepsy, Tuberculosis. Examination: She was Conscious & Coherent (GCS :15) & patient is Asthenic, Pale, Oral Cavity: Normal PR - 48/m, BP-90/60 mm hg (Supine position), RR - 26/m regular there is clinical evidence of B/L Pleural effusion Tenderness of right wrist & b/l PIP joints, neurologically patient has power of 1/5 in lower limbs, 4/5 in upper limbs, reflexes are absent in lower limbs and depressed in upper limbs, Rest of systemic examination was normal Investigations ECG: Bradycardia, Prominent U waves & Prolonged QTc interval. Serum electrolytes: Na+: 136 mEq/l, K+: 1.6 mEq/l, Cl -: 115 meq/l and HCO3 =10.8 mEq/l, Ca+2: 8.2mg/dl, Serum anion gap = 11.8 GRBS: 110mg/dl , ABG: pH = 7.24, TCO2 = 11.7 mmol/l, pCO2 = 25.1 mmHg, pO2: 118.9 mmHg, HCO3 =10.8 mmol/l, O2 sat = 97.9 %, & Hct:33% 24hr urine K+: 58 mEq/day 24- hour urine calcium: 98mg/day 24 hr total urinary protein: 511mg Urine pH: 6.18 Urine anion gap: + 89 Urine culture: Negative , Haemogram: Hb: 9gm %, TLC: 3,600/mm3, Platelets: 96000/mm3, ESR - 80mm/1st hr, Urine Acidification Test Confirmed dRTA HIV, HBsAg: Non Reactive, CXR PA: - B/L pleural Effusion, 2D Echo: pericardial effusion, EF: 62%, USG abdomen: moderate ascitis & b/l pleural effusion, Monteux test: < 5mm induration, Fluid analysis: Exudative - ADA levels (12 IU/dl: pleural Fluid & 22 IU/dl: ascitic fluid,), Thyroid profile: TSH: 8.14 úIU/ml , T3: 0.85 ng/ml T4: 5.4 úg/dl, Serum PTH: 33pg/ml . Serum LDH: 145 IU/l, Coomb's Test: -ve Anti ANA Ab: +ve & ANA profile: (Line Immuno assay) - Strongly positive for Anti sm, dsDNA, Nucleosomes, Rib p-prot Ab. Patients was Diagnosed as having SLE (as per ACR & SLICC Criteria) Discussion dRTA is associated with autoimmune diseases such as primary Sjögren syndrome (5%-12%) & SLE (1.8%-7%) Treatment & Followup Patient was kept on Steroids & Hydroxychloroquine at discharge, patient on followup after 40 days showed improvement & No episode of HPP till 3months followup

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Page 1: sle RTA.pdf

SLE - IT'S ATYPICAL CLINICAL PRESENTATION G.Ravi Kiran, Dr.V. Chandrashekar, Dr. Bikshapathi Rao

Department of General Medicine, MGM Hospital

Kakatiya Medical College, Warangal, Telangana

Introduction

Hypokalemic periodic paralysis (HPP) is a form of metabolic myopathy, characterized by hypokalemia,

acute flaccid paralysis, and potentially fatal episodes of muscle weakness through the involvement of the

respiratory muscles, and cardiac arrhythmias. It can be a primary disorder or secondary with causes like

renal tubular acidosis (RTA), Distal RTA (dRTA) is the most common form & it's association with

autoimmune diseases is well documented

Case Presentation

A 34 year old female patient presented with C/C

• Low grade fever & weight loss - 5 months,

• Weakness of both lower limbs - 15 days ,

• Inability to walk - 6 days,

Past History:

Patient had history of similar weakness of lower

limbs 1 episode - 45 days back, She is Not a Known

Case of HTN, DM, Asthma, Epilepsy, Tuberculosis.

Examination: She was Conscious & Coherent (GCS :15) & patient

is Asthenic, Pale, Oral Cavity: Normal

PR - 48/m, BP-90/60 mm hg (Supine position),

RR - 26/m regular there is clinical evidence of

B/L Pleural effusion

Tenderness of right wrist & b/l PIP joints,

neurologically patient has power of 1/5 in lower

limbs, 4/5 in upper limbs, reflexes are absent in

lower limbs and depressed in upper limbs,

Rest of systemic examination was normal

Investigations

ECG: Bradycardia, Prominent U waves &

Prolonged QTc interval.

Serum electrolytes: Na+: 136 mEq/l, K+: 1.6

mEq/l, Cl -: 115 meq/l and HCO3 =10.8 mEq/l,

Ca+2: 8.2mg/dl, Serum anion gap = 11.8

GRBS: 110mg/dl , ABG: pH = 7.24, TCO2 = 11.7

mmol/l, pCO2 = 25.1 mmHg, pO2: 118.9 mmHg,

HCO3 =10.8 mmol/l, O2 sat = 97.9 %, & Hct:33%

24hr urine K+: 58 mEq/day 24- hour urine calcium: 98mg/day

24 hr total urinary protein: 511mg

Urine pH: 6.18 Urine anion gap: + 89

Urine culture: Negative ,

Haemogram: Hb: 9gm %, TLC: 3,600/mm3,

Platelets: 96000/mm3, ESR - 80mm/1st hr,

Urine Acidification Test Confirmed dRTA

HIV, HBsAg: Non Reactive,

CXR PA: - B/L pleural Effusion,

2D Echo: pericardial effusion, EF: 62%,

USG abdomen: moderate ascitis & b/l pleural

effusion, Monteux test: < 5mm induration,

Fluid analysis: Exudative - ADA levels (12 IU/dl:

pleural Fluid & 22 IU/dl: ascitic fluid,),

Thyroid profile: TSH: 8.14 úIU/ml , T3: 0.85 ng/ml

T4: 5.4 úg/dl, Serum PTH: 33pg/ml .

Serum LDH: 145 IU/l, Coomb's Test: -ve

Anti ANA Ab: +ve & ANA profile: (Line Immuno

assay) - Strongly positive for Anti sm, dsDNA,

Nucleosomes, Rib p-prot Ab. Patients was Diagnosed

as having SLE (as per ACR & SLICC Criteria)

Discussion

dRTA is associated with autoimmune diseases such

as primary Sjögren syndrome (5%-12%) & SLE

(1.8%-7%)

Treatment & Followup Patient was kept on Steroids & Hydroxychloroquine

at discharge, patient on followup after 40 days

showed improvement & No episode of HPP till

3months followup