Rheumatolological Emergencies

Rheumatolological Emergencies

Most of the rheumatological disorders are insidious in onset and chronic in nature. However, clinicians do encounter emergencies in rheumatological practice. Common medical disorders may present as emergencies in a patient with rheumatic disease such as hypoglycaemia in a patient with diabetes mellitus (DM) and rheumatoid arthritis (RA) on hypoglycaemic agents and nonsteroidal anti-inflammatory drugs (NSAIDs). Problems involving multiorgans such as lungs, kidneys, heart, brain and joints may present in a catastrophic manner increasing the morbidity and mortality. Some of the rheumatological disorders may present as an emergency e.g. systemic lupus erythematosus (SLE) with cardiac tamponade, antiphospholipid antibody (Hughes) syndrome (APS) as a stroke.

Table 1 depicts a few rheumatological emergencies organ-wise and
Table 2. enlists emergencies caused by certain rheumatic disorders.

Table 1: Rheumatological emergencies organ-wise.
Central nervous system
• Encephalopathy
• Psychosis
• Cranial neuritis
• Seizures
Eye
• Optic neuritis
• Iridocyclitis
• Scleritis
Heart
• Pericarditis
• Myocarditis
• Endocarditis
Lungs
• Adult respiratory distress syndrome (ARDS)
• Pneumonitis (lupus, infective or hypersensitive)
Gastrointestine
• Gastritis
• Bleeding of perforated peptic ulcer
Kidney
• Acute glomerulonephritis
• Acute interstitial nephritis
• Diffuse arteritis
Blood
• Thrombocytopenia
• Aplastic anaemia
• Haemolysis
Skin
• Exfoliative dermatitis
• Steven Johnson syndrome
• Toxic epidermal necrolysis
Bone and joints
• Septic arthritis
• Osteoporotic fractures

Table 2: Rheumatological emergencies - disease-wise.

Rheumatoid arthritis
• Atlanto-axial dislocation
• Scleromalacia perforans
• Vasculitis
• Acute exacerbation of synovitis
• Infections

Seronegative sponyloarthropathy
• Iridocyclitis

Systemic lupus erythematosus
• Seizures, psychosis, encephalopathy
• Pericarditis, myocarditis, endocarditis
• Pneumonitis, ARDS
• Acute glomerulonephritis
• Vasculitis
• Hypertensive crisis
• Acute pancreatitis
• Polyserositis
• Infections

Antiphospholipid antibody syndrome
• Stroke
• Acute myocardial infarction
• Retinal vessel thrombosis
• Pulmonary embolism and infarction
• Thrombocytopenia
• Placental ischaemia and foetal loss
• Catastrophic APL syndrome

Vasculitis
• Cerebral vasculitis
• Optic neuritis
• Uveitis
• Mesenteric vasculitis
• Acute nephritis
• Hypertensive crisis

Systemic sclerosis and mixed connective tissue disease
• Digital vasculitis and ischaemia
• Scleroderma renal crisis

Inflammatory myositis (poly / dermatomyositis)
• Respiratory failure

Crystal-induced arthropathies
• Acute gout
• Acute interstitial nephritis

Arthritis related to infections
• Septic arthritis
• Reactive arthritis
Osteoporosis
• Fracture
Miscellaneous disorders
• Haemophilic arthropathy
• Rupture of Baker’s cyst

Drugs commonly used in various rheumatological disorders can also cause certain toxic effects presenting as emergencies (Table 3)

Table 3: Drugs - Emergencies

Nonsteroidal anti-inflammatory drugs
• Acute gastritis
• Perforated / bleeding peptic ulcer
• Acute enteritis
• Analgesic nephropathy
• Acute interstitial nephritis
• Hypersensitivity reactions
• Thrombocytopenia
• Pancytopenia
• Steven Johnson syndrome
• Erythema multiforme
• Toxic epidermal necrolysis
• Acute interstitial nephritis

Glucocorticoids
• Psychosis
• Acute Addisonian crisis due to withdrawal

Disease- modifying antirheumatic drugs
• Hypersensitivity reactions
• Thrombocytopenia
• Aplastic anaemia
• Exfoliative dermatitis
• Steven Johnson syndrome

Pathogenesis
Direct involvement of vital organs by the disease process can present as an event e.g. rheumatoid lung, neurolupus, scleroderma kidney.

A common basis for most emergencies in rheumatological disorders is necrotising vasculitis with inflammation leading to target organ ischaemia and vital organ failure. Vascular occlusion and target organ damage may also occur due to thrombosis in both arterial and venous system as seen in APS syndrome.

Principles of Evaluation and Management
Assessment of a patient presenting with a rheumatological emergency is based upon thorough history and clinical examination. The primary aim of the attending physician is to save life and to prevent irreversible organ damage by instituting suitable and aggressive therapy.

A team approach involving anesthetists, nephrologists, cardiologists and other relevant specialists as required in an individual case is essential for management of any rheumatological emergency.
Systemic Lupus Erythematosus
Institution of aggressive therapy beginning with high dose of glucocorticosteroids (GCS) should be used whenever a patient has life-threatening SLE that is likely to respond to steroids (Table 4).1

Infections must be carefully excluded before instituting or increasing GCS therapy. Consideration must also be given to the presence of co-morbid conditions such as hypertension diabetes mellitus, obesity, osteoporosis and psychiatric disorders.
Table 4: Life threatening manifestations of SLE -responses
to glucocorticoids (GCS)
a. Manifestations usually responsive to high doses of GCS
• Severe dermatitis of subacute cutaneous lupus
• Acute polyarthritis
• Polyserositis
• Myocarditis
• Lupus pneumonitis
• Glomerulonephritis
• Haemolytic anemia
• Thrombocytopenia
• Diffuse CNS syndrome
• Lupus crisis (high fever and prostration)
b. Manifestations not often responsive to GCS
• Thrombosis
• Scarred end stage renal disease
• Membranous glomerulonephritis
• Psychosis not related to SLE

Initial therapy should be either high dose daily GCS (1-1.5 mg/kg) given in divided doses or IV methyl prednisolone pulses (500-1000 mg/d for 3 days) followed by oral prednisolone. Combining GCS with cytotoxic drugs such as cyclophosphamide and azathioprine is superior to GCS alone in controlling acute severe SLE, reducing irreversible tissue damage and prolonging survival.2

Cyclophosphamide is typically given with GCS either orally (1-3 mg/kg daily) or intravenously (0.5 to 1.0 gm/m2 of body surface area). The most commonly used intravenous regimen consists of monthly infusions for a course of 6 months followed by infusions every 3-6 months.3

New therapies for aggressive SLE include intravenous gammaglobulins,4 mycophenolate mofetil (blocks de novo guanosine synthesis)5 and immunoablation with autologous stem cell transplantation.6 Despite early reports of efficacy comparative randomized trials have failed to show the superiority of apheresis-cyclophosphamide over IV cyclophosphamide alone in active lupus nephritis.7 Trials with B-lymphocyte depletion by rituximab are showing promising results in SLE.8
Antiphospholipid (Hughes) Syndrome (APS)
APS is a noninflammatory autoimmune disease. The critical pathologic process is thrombosis, which results in most of the clinical features suffered by the patients. Any organ and any size of vessel can be affected leading to some emergencies. Clinical manifestations of APS associated with central nervous system include arterial thrombotic events, psychiatric features and non-thrombotic neurological syndromes. Stroke at a younger age is the most common manifestation of APS associated with antiphospholipid antibodies(apl).9,10,11 The most important cardiac manifestations presenting as emergencies in APS are valve disease and coronary artery disease.12 Pulmonary manifestations of APS include pulmonary hypertension. Some cases of diffuse alveolar haemorrhage have also been described.13 Haematological manifestations such as thrombocytopenia (most frequent), obstetric manifestations with maternal and foetal complications are well described with APS. Renal involvement includes thrombosis of glomerular capillary, thrombotic microangiopathy, cortical necrosis, renal artery thrombosis and renal vein thrombosis.14


Recurrent arterial and venous thrombosis can be prevented with warfarin anti-coagulation to an INR International normalized ratio) of = 3. Lifelong anticoagulation of patients is necessary in most of the situations. Catastrophic vascular occlusion leading to multi-organ failure is usually sudden, diagnostically confusing and immediately life threatening. Most effective treatment combines full dose anticoagulation, high dose GCS, plasmapheresis and intravenous immunoglobulins.15
Vasculitides

The vasculitides are heterogenous group of uncommon diseases characterized by blood vessel inflammation and necrosis. These conditions often have overlapping clinical and pathologic manifestations. Most of the vasculitides are systemic and may involve multiple organs. Primary vasculitis (when no underlying diseases were found) encompass vasculitis affecting predominantly large, medium and small sized blood vessels. Secondary vasculitis include vasculitis secondary to rheumatic diseases such as RA and SLE and vasculitis secondary to non-rheumatic diseases like sarcoidosis, malignancy and severe bacterial infections.16

Apart from some uncommon acute events (Table-5),
rare forms of emergencies are encountered in the patients with vasculitides e.g. cerebral infarction in Wegner’s granulomatosis.17

Management has to be individualized depending upon the organ involved and type of vasculitis.

Not uncommonly the initial management of these emergencies could be based on a presumptive diagnosis.

Therapy should not be deferred pending confirmation of diagnosis by laboratory tests. The treatment involves GCS, cyclophosphamide, azathioprine, methotrexate, cyclosporine and immunoglobulins based upon the emergency situation.18

Sai Rheumatology Centre
HNO -3-4-526/1/A, Barkatpura,Near Bank Of Baroda,Opposite To Old Post Office Lane
Hyderabad, Andhra Pradesh 500027
040 2755 0083‎
09247778944‎ - Mobile
09392589931
Email‎: sairheumatology@gmail.com

Specialised Clinic In Arthritis

What is the Rheumatology ?

The Musculoskeletal disorder is (Rheumatology)

1.Difficulty in walking or setting.

2.Muscle pains and morning stiffness restricted joint movements

3.Neck pain or stiffness

4.Blow back pain ,Spinal pain aching limbs and bone pains ,Osteoporosis .

5.Ankle pain, Hip pain, Wrist pain, Joints pain,Sweiiling,Redness and Warmth .

6.Decrease in appetite , weight loss fatigue low-grade fever .

Common rheumatic diseases
OSTEOARTHRITIS is one of the most common types of arthritis. It affects the cartilage, which cushions the ends of the bones. Gradually the cartilage thins and may even break down, leaving the ends of the bone unprotected. Osteoarthritis occurs most commonly in the fingers and weight bearing joints, including the knees, feet, hips and back.

RHEUMATOID ARTHRITIS is a disease that causes inflammation of the joints, usually in the hands, feet and knees. The exact cause is unknown. The resulting inflammation leads to swelling, pain and stiffness in multiple joints simultaneously. In the long term if this is left untreated it can lead to damage and deformity of the joint.

OSTEOPOROSIS is a common disease in which bones become thin and can break more easily.To prevent osteoporosis and improve the strength of your bones it is important to make sure you get enough calcium and Vitamin D. It is also important to do regular weight bearing exercise, such as walking.

Rheumatologists are experts in managing these and other autoimmune and connective tissue diseases such as systemic lupus erythematosus, scleroderma, inflammatory myositis and vasculitis.

WHAT WILL HAPPEN WHEN I SEE THE RHEUMATOLOGIST

THE DIAGNOSIS

Your rheumatologist is specially trained to do the detective work necessary to discover the cause of your symptoms. He or she will usually ask you to explain the

history of the problem and will undertake a physical examination. Additional investigations such as blood tests, X-rays and scans may also be needed.

INFORMATION AND ADVICE

Once a diagnosis is made, your rheumatologist will explain the nature of your illness and what you might expect in the future. This is an important step,

particularly for illnesses which might affect you over a long period.

TREATMENT

With an accurate diagnosis and a shared understanding of your illness, you and your rheumatologist can work together to design a treatment program aimed at managing pain, reducing inflammation and ensuring your quality of life.

ONGOING MANAGEMENT

Depending on the nature of your illness, you may need to see your rheumatologist regularly for ongoing management. Alternatively you may be treated by your

general practitioner, with the rheumatologist on hand for specialist advice.

SO HOW WILL MY CONDITION BE TREATED?

As your rheumatologist will explain, there are a number of treatment options available including:

PHYSICAL THERAPY

GENERAL SUPPORTIVE CARE

DRUG TREATMENT AND SURGERY

He or she will select the best treatment combination for you, depending on the exact nature of your illness and your other individual needs.

In treating and managing your illness, your rheumatologist will work closely with your GENERAL PRACTITIONER as well as other skilled professionals to ensure you get the best possible care:

The PHYSIOTHERAPIST is trained in the physical treatment of rheumatic disorders and uses a combination of exercise, special treatment techniques and advice.

The OCCUPATIONAL THERAPIST provides advice on how to get on with day-to-day activities such as working, dressing and getting out and about.

The PSYCHOLOGIST OR SOCIAL WORKER offers advice and support to help patients and families cope with the changes that illness might bring.

NURSES, PODIATRISTS AND DIETITIANS may also form part of the team involved in your treatment.

PROCESS…….............................

A Rheumatologist is a specialist physician who has expertise in diagnosing and treating diseases of the joints, muscles and bones. Some times the term rheumatic disease is used to describe this group of diseases.

The more common rheumatic diseases include:

JOINT DISEASE

Arthritis

Gout

Spinal problems

Osteoarthritis

MUSCLES & TENDONS

Fibromyalgia

Polymyalgia rheumatica

Tendonitis

BONE DISEASE

Osteoporosis

Paget’s disease

here are also a number of rarer rheumatic conditions which can affect other parts of the body including the skin, hair and internal organs. These include “lupus”, myositis, vasculitis and scleroderma.

WHERE DO I GO FOR MORE INFORMATION ?

Support research into arthritis and other musculoskeletal disorders :

This Site provides answers to some of the commonly asked questions.

If you would like further information please speak with your rheumatologist.

If you wish to find a rheumatologist in your local area,

Consult the directory on the web site:

For more general information about arthritis and related diseases contact :

SAI RHEUMATOLOGY CENTRE

# 3-4-526/1/A , BARKATHPURA , HYDERABAD .

Consultation by Appointment

Ph:0091- 92477-78944 , 9392589931
E-mail : rheumatologyinhyd@gmail.com