Diagnostic Criteria For

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1. Malar rash: fixed erythema, flat or raised over the malar area, that tend to spare nasolabial folds

2. Discoid rash: erythematous raised patches with adherent keratotic scaling and follicular plugging

3. Photosensitivity: skin rash as a result of exposure to sunlight

4. Oral or vaginal ulcers: usually painless

5. Arthritis: nonerosive. involving two or more peripheral joints with tenderness, swelling, and effusion

6. Serositis: usually pleuritis or pericarditis

7. Renal involvement: persistent proteinuria or cellular casts

8. Neurologic disorder: seizure or psychosis

9. Hematologic disorder: hemolytic anemia or leukopenia (<4000/mm3) on two or more occasions, or lymphopenia <1500/mm3) on two or more occasions, or thrombocytopenia (<100,000/mm3)

10. Immunologic disorder: positive LE (lupus erythematosus) cell preparation or positive anti-dsDNA (anti-double-stranded DNA) or positive anti-Sm (anti-Smith antibody)

11. Antinuclear antibody (ANA): positive ANA

Our patient has serositis (pericarditis), oral ulcers, hematologic disorders (leukopenia, lymphopenia, thrombocytopenia), arthritis, and renal involvement (hematuria)—she clearly meets the criteria for SLE. Although the patient in the scenario is like most lupus patients in that she sought medical attention because of the pain of arthritis or serositis, both of these problems are generally manageable or self-limited. The arthritis is generally nonerosive and non-deforming, and the serositis usually resolves spontaneously without sequelae. The major complication of SLE usually is related to renal involvement, which can cause hypertension, chronic renal failure, nephrotic syndrome, or end-stage renal disease. In the past, renal disease was the most common cause of death of SLE patients, but now it can be treated with powerful immunosuppressants, such as high-dose corticosteroids or cyclophosphamide. Other serious complications of lupus include central nervous system (CNS) disorders, which are highly variable and unpredictable and can include seizures, psychosis, stroke syndromes, and cranial neuropathies. In addition to renal failure and CNS involvement, the most common causes of death in SLE patients are infection (often related to the immunosuppression used to treat the disease) and vascular disease, for example, myocardial infarction.

Comprehension Questions

[18.1] A 48-year-old man is admitted to the hospital for chest pain. On examination, a pericardial friction rub is noted. He has end-stage renal disease as a consequence of diabetes. Which of the following is the best treatment?

A. NSAIDs

B. Dialysis

C. Steroids

D. Kayexalate (sodium polystyrene sulfonate)

[18.2] Which of the following is the most likely outcome following an episode of acute pericarditis?

A. Myocardial infarction

B. Tamponade

C. Constrictive pericarditis

D. Recovery without sequelae

[I8.3[ A 25-year-old woman with symmetric arthritis of the proximal inter-phalangeal (PIP) and metacarpophalangeal (MCP) joints is noted to have thrombocytopenia, hematuria, and a pleural effusion. Which of the following is the most likely diagnosis?

A. Systemic lupus erythematosus

B. Rheumatoid arthritis

C. Gout

D. Reiter syndrome

Answers

[18.11 B. Dialysis is the best treatment for pericarditis associated with renal failure.

[ 18.2] D. Acute pericarditis usually resolves without sequelae.

[18.3] A. SLE is associated with symmetric arthritis and serositis (usually pleuritis or pericarditis).

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