RHEUMATOLOGICAL EMERGENCY 2019

1. Dr Shivaom Chaurasia
Internal Medicine
Resident

2.  According to the American Medical Association (AMA),
◦ emergencies are defined as those situations that place the life of the
patient or an organ function in immediate danger.
 World Health Organization (WHO)
◦ emergency refers to a case where failure to provide care may lead to
the death of the patient in a matter of minutes to hours.
 True emergencies are those that are life threatening for the
patient with mortality rates of around 50 % even when timely
intervention is provided.

3.  RHEUMATOID ARTHRITIS-
◦ Septic Arthritis, Atlantoaxial Dislocation, Acute Upper Airway Obstruction
 SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
◦ Acute Transverse Myelitis, Cerebrovascular Accident
 VASCULITIS
◦ Alveolar Haemorrhage and Pulmonary Renal Syndrome(PRS), Visual Impairment
in Giant Cell Arteritis (GCA), Acute Mesenteric Vasculitis, Mononeuritis Multiplex
 SYSTEMIC SCLEROSIS
◦ Scleroderma Renal Crisis
 INFLAMMATORY MYOPATHIES
◦ Respiratory Muscle Weakness, Cardiac Disease
 CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME

4.  Septic Arthritis
◦ Caused by pyogenic bacteria.
◦ Infection spreads through the bloodstream, or surgery, injection, or
injury.
◦ Causative organism -----usually Staphylococcus or Streptococcus.
◦ Adults---------gonococci and mycobacteria.
◦ Intravenous drug users and immunocompromised ---gram-negative
bacteria .

5.  Symptoms----
◦ Severe pain and swelling in the affected joint
◦ Fever with chills
 Septic arthritis in the hip
◦ pain in the groin area that becomes much worse if the patient tries to
walk.

6.  Synovial fluid analysis
◦ TLC---high, and
◦ Culture
 Radiographs ----to assess any joint damage.
 Antibiotics should be started and should be continued for at
least six weeks.

7.  Surgical drainage----
◦ reserved for septic arthritis of the hip.
 Splinting----pain relieve.
 After a period of rest, gentle exercise to prevent stiffness
should be started.
 Recovery is usually good
 Osteoarthritis or deformity can occur.

8. Atlantoaxial Dislocation
◦ Anterior subluxation (most common); vertical subluxation of the dens
may also occur.
◦ Symptoms
 paraesthesia, numbness, and muscular weakness.
◦ In severe cases
 spastic paralysis, paraplegia, tetraplegia, sensory loss and also loss of
bladder control, faecal incontinence, and syncope.
◦ Physical examination
 upper motor neuron weakness, hyper reflexia, and a positive Babinski’s
sign.

9.  Magnetic resonance imaging (MRI)
◦ assessment of spinal cord damage.
 Treatment
◦ Immediate immobilization (Neck collar),
◦ NSAIDs and
◦ Simple neck traction.
 A single horizontal or vertical spinal dislocation without significant
neurologic features requires no surgical intervention.
 Neurologic symptoms and signs of cord compression--- strong
indication for neurosurgery.

10. Acute Upper Airway Obstruction
 Cricoarytenoid arthritis can occur in RA.
 Rheumatoid nodules (larynx and trachea)
◦ erode through local structures.
◦ If large, the nodules can cause dyspnoea (obstruction of the larynx
during acute infections).
 Symptoms ----
◦ dysphonia
◦ aspiration
◦ acute airway compromise.

11.  Indirect laryngoscopy (acute phase)
◦ erythema and swelling of the arytenoid mucosa.
 Direct laryngoscopy (definitive)---
◦ cricoarytenoid joint fixation.
 HRCT (IOC)
◦ assess cricoarytenoid involvement.
 Management
◦ Tracheostomy for an acute obstruction.
◦ Medical treatment - corticosteroids at the earliest.
◦ Periarticular local steroid injection.

12. Acute Transverse Myelitis
◦ Presents as a rapidly progressive motor, sensory and autonomic
dysfunction.
◦ Increased risk in presence of APLA syndrome.
◦ Pathogenesis
 related to vascular thrombosis, or to direct interaction between
antiphospholipid antibodies and membrane phospholipids at the spinal cord
level.

13.  Clinical presentation
◦ sudden onset of leg weakness or sensory loss and/or bowel or bladder
dysfunction.
 Evolution of the neurodeficit ----complete 1 to 2 days.
 Thoracic spinal cord is most commonly involved followed by
lumbar and cervical.

14. Cerebrovascular Accident
 Vasculitis, local thrombosis or emboli.
 Emboli can occur as a result of Libman-Sacks endocarditis.
 Strokes may be secondary to treatment of SLE
 Investigations
◦ Cerebrospinal fluid (CSF) analysis to rule out infection.
◦ Low levels of C4.
◦ MRI superior to conventional computed tomography (CT).

15.  Corticosteroids
◦ Methylprednisolone (0.5 to 1g per day) intravenously for 3 to 5 days
followed by oral prednisolone 1 mg/kg.
 Intravenous cyclophosphamide (0.5 to 1 g)
◦ effective in serious forms of central nervous system manifestations.
 With APLA----anticoagulation is necessary.

16. Alveolar Haemorrhage and Pulmonary Renal Syndrome (PRS)
◦ Alveolar haemorrhage is a medical emergency and occasionally is the
initial presenting manifestation.
◦ Pulmonary-renal syndrome is diffuse alveolar haemorrhage and
glomerulonephritis occurring simultaneously.
◦ Haemoptysis is the most classic finding in combination with
peripheral oedema and haematuria or other signs of
glomerulonephritis.

17.  Pulmonary infiltrates may be diffuse or patchy, and usually are
perihilar, sparing the apices
 Renal pathology
◦ small-vessel vasculitis resulting in a form of focal proliferative
glomerulonephritis.
 Major Causes
◦ Goodpasture’s syndrome
◦ Wegener’s granulomatosis,
◦ Microscopic polyangiitis,

18. Investigations-
 Urinalysis- initial test
◦ evidence of haematuria and red cell casts (suggesting glomerulonephritis).
 Diffusion capacity of carbon monoxide (DLCO) may be increased.
 Bronchoscopy
◦ exclude infection and to document the presence of blood
 Hemosiderin-laden macrophages provide proof that bleeding has
occurred within the lung.

19.  Serum antibody testing
◦ Antiglomerular basement membrane antibodies -Goodpasture’s
syndrome,
◦ Antinuclear antibodies (ANA), and anti-deoxyribonucleic acid antibodies
-SLE,
◦ Anti-neutrophil cytoplasmic antibodies (ANCA) directed against
proteinase-3 (PR3-ANCA) - Wegener’s granulomatosis,
◦ Perinuclear ANCA (p-ANCA) in microscopic polyangiitis.
 Definitive diagnosis
◦ lung biopsy and/or renal biopsy.

21.  Standard therapy
◦ pulse intravenous methylprednisolone (500 to 1000 mg once a day for 3 to 5
days).
◦ As life-threatening features subside, the dose can then be reduced to 1
mg/kg, prednisone tapered gradually over the next 12 weeks.
 Cyclophosphamide
◦ added at a dose of 0.5 to 1 g/m2 administered intravenously as a monthly
pulse or 1 to 2 mg/kg per day orally.
 Maintenance therapy –
◦ low-dose corticosteroids coupled with cytotoxic agents.
 Mechanical ventilation with high positive end expiratory pressure may
be required.

23. Acute Mesenteric Vasculitis
◦ Uncommon complication of systemic vasculitis.
◦ Bowel ischaemia can lead to perforation and haemorrhage-high
mortality.
◦ Onset may be acute and severe (e.g. mesenteric infarction) or chronic
due to mesenteric ischaemia.
◦ Chronic mesenteric ischaemia
 manifested by a variety of symptoms including abdominal pain after eating
(‘intestinal angina’), weight loss, nausea, vomiting and diarrhoea.

24.  Ultrasonography
◦ oedematous bowel loops.
 Gastroscopy and colonoscopy
◦ signs of ischaemia and ulceration.
 Common CT findings
◦ dilated bowel, focal or diffuse bowel-wall thickening, abnormal bowel-
wall enhancement, mesenteric oedema, engorgement of mesenteric
vessels and ascites.
 CT Angiography
◦ Vascular thromboembolism and aneurysms.

25.  Aggressive anti-inflammatory immunosuppressive
treatment should be initiated.
 Treatment of choice
◦ high-dose methylprednisolone
 In non responding patients and with severe haemodynamic
compromise
◦ surgical management is required.

26. Scleroderma Renal Crisis
◦ Scleroderma renal crisis presents with accelerated hypertension
accompanied by acute kidney injury and progressive failure.
◦ Acute life-threatening complication of SSc occurs in 10–15% of patients,
generally within 4 years of disease onset.
◦ Markedly elevated plasma renin activity and dramatically rising
creatinine is found in most cases.

27.  Vasospasm occurring on pre-existing renal arteriolar disease causes
excessive renin release.
 Angiotensin II induce further renal cortical ischaemia and fixed
loss of cortical blood flow and necrosis.
 Vascular injury in the form of intimal disruption, fibrin
deposition and microangiopathic haemolysis .

28.  Clinical Features-
◦ Headache, blurred vision, congestive heart failure, and pulmonary edema
may accompany elevation of blood pressure.
 Urinalysis
◦ mild proteinuria, granular casts, and microscopic hematuria
 Poor outcome
◦ Palpable tendon friction rubs
◦ pericardial effusion
◦ Unexplained anemia, thrombocytopenia
◦ Oliguria
◦ Creatinine >3 mg/dL at presentation
◦ Biopsy findings - vascular thrombosis and glomerular ischemic collapse.

29.  Treatment
◦ Avoidence of nephrotoxic drugs
◦ Short acting Angiotensin converting enzyme (ACE) inhibitors
◦ Patient with persistant HTN addition of ARB, CCB, endothelin-1
receptor blocker etc can be used
◦ Dialysis in 2/3rd cases may be necessary
◦ Kidney transplant appropriate if unable to discontinue dialysis after 2
years.

30. Respiratory Muscle Weakness
◦ Both inspiratory and expiratory muscles may be affected, including the
diaphragm.
◦ Bulbar weakness
 hoarseness or dysphonia, dysphagia with regurgitation of liquids.
◦ Pharyngeal and tongue
 increases the risk of aspiration.

31.  Muscle biopsy
◦ confirm the diagnosis.
 Arterial blood gases
◦ hypoxia with carbon dioxide retention.
 Chest radiograph
◦ exclude infection.
 Outcome is usually good with most patients regaining power
to normal.

32.  Intravenous Methylprednisolone (1g per day) for 3 to 5 days
followed by oral prednisolone 1mg/kg per day.
 IVIG or plasmapheresis
◦ severe nonresponding disease.
 Biological agents
◦ Rituximab may be used.
 Mechanical Ventilation
◦ Increasing hypoxia, neck muscle weakness, paradoxical breathing

33. ◦ Catastrophic APS (CAPS) is a life-threatening rapidly progressive
thromboembolic disease involving simultaneously three or more
organs.
◦ widespread vascular occlusion ( in medium or small sized arteries) in
multiple organs ( > 3 organs) in few days.
◦ Mortality rate 50%

35. Treatment of catastrophic APS:
 Hospitalization
 Anticoagulation
 Plasmapharesis
 IVIG
 Corticosteroids
 Cyclophosphamide (especially in SLE-associated CAPS).

36.  Spectrum of emergencies varies from patient to
patient.
 Superimposed infections, disease flares and adverse
reaction to drugs can contribute to morbidity and
mortality.

37.  Rheumatologic emergencies (DOI 10.1007/s10067-015-
2994-y):
◦ Luis Arturo Gutiérrez-González
 Harrison’s Principles of Internal Medicine Twentieth Edition
 API Textbook of Medicine

38. Thank you