Case report : SLE with Situs Inversus Totalis

22years single female with clear medical background presented

with 2 months history productive cough and bilateral hand joint
pain , wound at buttock for 1 month . patient condition started
2 months prior to admission with productive cough
progressive , recurrent through day at night associated with
yellowish sputum which is copious , and offensive sometimes
but no postural relationship , there is no hemoptysis , there is
no shortness of breath , chest pain , wheezes and lower limb
edema,
The wound in her buttock started 1 month ago gradually
increasesed in size , it was painful , covered with pus , no other
wounds .
No rhinorrhea , stuffy nose , nasal block or headache
Patient also had bilateral hand joint pain affecting PIP and MCP
not associated with swelling , morning stiffness , all other joints
are spared , she had photosensitive facial rash and marked hair
loss , there is no oral or nasal ulcers .
Patient also complained of muscle pain and weakness affecting
upper and lower limbs ; she cannot comb her hair or stand
from setting position.
She has fever which is intermittent of moderate grade and
associated with sweating and rigors , there is noticeable weight
loss and fatigue

Systemic review was unremarkable

PMH:
Apart from repeated chest infections for the last 2 years for
which she received oral antibiotic no significant past medial
history , no history of TB , no previous hospital disease or
surgical operations

FH:
No family history of similar conditions , no family history of
rheumatological , autoimmune disease or respiratory
diseseases

DH :
Not known allergic to any mediations not on long term
medications
OBS and GYN :
menarche at the age of 11 , since her illness began she had
irregular cycle , she has amenorrhea for last 2 months
Social history :
Low socioeconomic status , she has no no health insurance ,
there is no bad habits

Clinical examination at presentation

Patient looks unwell emaciated pale not jaundiced
There is butterfly rash on her face
GCS 15\15
BP 100/70
PR 88 beat/min
O2 saturation 94
Patient lying on cardiac bed tachypnic
CVS : normal precordium on inspection but apex is not palpable
on left side but clearly felt on the Rt side , normal s1 , s2, no
added sounds , murmurs and lower limb edema
Chest : patient is tachepneic distressed both side move
equally ,apex on rt side trachea mildly deviated to rt side ,
normal TVF , normal chest resonance on percussion ,
auscultation revealed decreased air entry on both lower zones ,
bronchial breathing and coarse creps altered with coughing no
wheezes

Abdomen And CNS examination was un remarkable

Examination of hand :
There is clubbing grade 3, there is no deformity ,joint
swelling ,tenderness or hotness
There is no evidence of synovitis
There is generalize muscle wasting power is grade 3+
proximally in both upper and lower limbs , distally power is
grade 5
There is grade 4 bed ulcer at left buttocks , the bone is
exposed , with pus discharge

Investigations:

CBC

TWBC 7.8 ×103/ul

HB 9.2 g/dl
MCV 60 fl
MCH 20 pg
MCHC 33 g/dl
PLT 800 × 103 /ul
Neutrophils 5.8 × 103/ul
Lymphocytes 1.4 × 103/ul

RFT and electrolyte

Urea 23 mg/dl
Creatinine .7 mg/dl
Na 136 mmol/l
k 3.6 mmol/l

LFT and enzymes

Total protein 3.5 g/dl

albumin 1.3 g/dl
Bilirubin total .3 mg/dl
Bilirubin direct .1 mg/dl
AST 18 i.u /l
ALT 17 i.u /l
ALP 250 u/l

Inflammatory Markers

ESR 95 mm/hour
CRP 17 mg/l

ANA Profile 3 :
Positive for RNP /Sm , Ro 52 , weakly positive for Nucleosome

RBS : 113

Bed sore swab culture results :

Proteus species resistant to: Cefotaxime , Ceixime , Ceftriaxone
, Cefuroxime , Amoxicillin , Amoxclav
Sensitive to : Meropenem , Gentamycin
CHEST X ray
X rays sinuses
HRCT chest
Echocardiography :
Dextrocardia, EF 80%, normal pulmonary artery pressure ,

Disease Course:
Patient was admitted for 1 month received HCQ 200 OD
Plastic surgeon consulted adviced regular dressing and planned
graft for bed sores and optimization of her health status prior
to surgery , she received injectable antibiotics according to
culture and sensitivity and protein formula her condition
improved fever subsided , her hair grew back but she failed to
gain weight , she stayed in hospital for over 1 month , upon the
insistence of the patient and her family we had to discharge her
and arranged for her to be seen plastic surgery department in
Omdurman military hospital who were willing to take her under
their care and do the graft surgery for her for free as an
academic case but unfortunately Zakia didn’t go to OMH ,
despite several call from my side and stalling from her family ,
they had deeply rooted belief she is being possessed by jinn ,
and medicine won’t be able to save her and took her to
traditional healer , sadly Zakia passed away 2 weeks ago may
Allah have blessing on her soul
Situs inversus

congenital condition in which the major visceral organs are

reversed or mirrored from their normal positions. The normal
arrangement of internal organs is known as situs solitus.
is found in about 0.01% of the population, or about 1 person in
10,000. In the absence of congenital heart defects, individuals
with situs inversus are homeostatically normal, and can live
standard healthy lives, without any complications related to
their medical condition. There is a 5–10% prevalence of
congenital heart disease in individuals with situs inversus
totalis, most commonly transposition of the great vessels. Situs
inversus is generally an autosomal recessive genetic condition,
although it can be X-linked or found in identical "mirror image"
twins1.
Diagnosis of situs inversus can be made using imaging
techniques such as x-ray, ultrasound, CT scan, and magnetic
resonance imaging (MRI). Any potential treatment for situs
inversus would involve a complete and highly invasive surgical
rearrangement of the internal viscera of the patient. Such a
procedure is unnecessary, given that situs inversus rarely
causes any additional symptoms. No treatment, medical or
surgical, is prescribed for situs inversus patients1 .

Reviewing the published literature revealed only one similar

case of patient of Mixed connective tissue disease associated
with Situs Inversus Totalis but we never came across a
published of SLE with Situs Inversus Totalis .
1. Dyskinesia PC, Atresia B. Situs Inversus Primary
Ciliary Dyskinesia Primary Ciliary Dyskinesia.
2006.