continuous medical education

2. SLE is a remitting and relapsing inflammatory autoimmune
illness, with a variety of different presentations.
The word lupus (from the Latin word for wolf) is attributed
to the thirteenth century physician Rogerius, who used it to
describe erosive facial lesions that were reminiscent of a
wolf's bite.

3.  Fast Facts
 Sun exposure can lead to lupus flares.
 Involvement of the kidneys or/and the brain is
the most serious manifestation of lupus.
 Treatment depends on the organs involved.
 Lupus can affect children; in fact, 20% of
patients with lupus are children.
 Lupus occurs ten times more often in women
than in men.

8. The new systemic lupus erythematosus
classification criteria of the American College of
Rheumatology and the European League
Against Rheumatism are based on a point
system.

10.  Infection.
 Common sites of infection are urinary tract,
lungs, joints, central nervous system, abdomen
and skin.
 Susceptibility to infection is due to immune
dysfunction, as manifested by phagocytic
dysfunction, lymphopenia (CD4 +
lymphocytopenia), decreased cytokine
production, decreased immunoglobulin
production, impaired functioning of
complement system and functional asplenia.

11.  Upper airways obstruction due to
 Laryngeal inflammation
 Epiglottitis
 Vocal cord paralysis
 Retropharyngeal abscess
 These need early recognition and urgent
intubation.
 SLE patients are more susceptible to develop
post intubation stricture

12.  Lupus pneumonias
 Pulmonary haemorrhage
 Pulmonary hypertension
 Shrinking lung syndrome
 Pulmonary embolism
 Treatment is with high dose
glucocorticosteroids ± antibiotics ±
immunosuppressive therapy.
 Mortality is high (upto 50%).

13.  Premature coronary artery disease (CAD)
with myocardial infarction
 Vasculitis
 Myocarditis
 Pericarditis
 Valvular affection

14.  Lupus cerebritis
 Cerebrovascular accidents
 Aseptic meningitis
 Seizures
 Transverse myelitis
 Psychosis
 Extrapyramidal syndrome
 GBS

15.  Bowel perforation
 Pancreatitis
 Bowel ischaemia
 Rupture of hepatic artery aneurysm
 Patients with active SLE are more likely to develop
vasculitis of abdominal vessels. Bowel ischaemia and
perforation warrant early diagnosis and emergency
laparotomy, while simultaneously continuing treatment
for active lupus. Pancreatitis poses difficult therapeutic
problem as steroids can cause pancreatitis. However, in
most patients, pancreatitis is a manifestation of active
lupus and patients need to be treated with steroids,
apart from usual measures. Mortality is high.

16.  Autoimmune haemolytic anaemia
 Thrombocytopenia
 APL syndrome
 Thrombotic thrombocytopenic purpura

17.  Rapidly progressive glomerulonephritis is an
important renal emergency. Early diagnosis
with energetic treatment (plasmapheresis,
steroid & cyclophosphomide pulses) can
prevent development of irreversible renal
damage

18.  There is an increase in pregnancy related
complications such as
 Toxaemia of pregnancy,
 Neonatal lupus,
 intrauterine growth retardation (IUGR),
 heart block,
 Abortion and
 Premature delivery are the hazards to the
foetus.

19.  An active disease is not an indication for
MTP. Prednisolone even in large doses is safe
as it does not cross placental barrier. For
treatment of foetal affection (evolving heart
block) dexamethasone or betamethasone is
to be used (cross placental).
 Anti-Ro antibody is the risk marker. Such
pregnancies need close monitoring with
periodic foetal echocardiograms.

21.  APL antibodies cause repeated abortions in
second trimester, toxaemia, IUGR, premature
labour & still births. Anticoagulation along
with aspirin are indicated throughout the
pregnancy.

22.  There is no cure for lupus, as it is a chronic disease.
The treatment goals are to suppress the overactive
immune system and ultimately induce remission and
prevent permanent organ damage.
 Antimalarial drugs: Hydroxychloroquine (Plaquenil), is
recommended for every patient with lupus.
 Corticosteroids and immune suppressants: Patients
with serious or life-threatening problems such as
kidney inflammation, lung or heart involvement, and
central nervous system symptoms need additional,
stronger treatment.This may include high-dose
corticosteroids such as prednisone and other drugs
that suppress the immune system.

23. Immune suppressants
azathioprine (Imuran),
Mycophenolate mofetil (Cellcept),
Methotrexate,
Cyclophosphamide (Cytoxan) and
Rituximab (Rituxan).
Other medications help control pain, including nonsteroidal anti-inflammatory
drugs (NSAIDs) like ibuprofen or naproxen.
Biologics: In 2011, the FDA approved a biologic, belimumab (Benlysta), for the
treatment of active SLE in adult patients and it was approved in 2019 for use in
children. Benlysta has shown to be effective in mild forms of lupus and it is the first
new drug approved for lupus since 1955.

24.  It is clear that SLE covers the whole gamut of
internal medicine.Treating SLE emergencies
can be extremely taxing and sometimes
frustrating; but the rewards are a million
times more satisfying.

25.  Conclusion
 It is clear that SLE covers the whole gamut of
internal medicine.Treating SLE emergencies
can be extremely taxing and sometimes
frustrating; but the rewards are a million
times more satisfying.