22 Years Old Female With Rash And Joint Pain

A 22 years old female presents to the clinic with a history of fatigue, on and off fever and pain in  her joints. On examination she has a characteristic butterfly rash over the cheeks and nose.

Case Discussion:

Systemic Lupus Erythematosus (SLE) :
A multisystem chronic inflammatory disease that is secondary to ANA complex formation and deposition. Patients may experience acute flare ups of their symptoms. SLE is generally primary, but sometimes occur secondary to drug use.

Drugs That Can Cause SLE:

• Hydralazine
• Procainamide
• Penicillamine

The drug induced SLE is reversible. 

Clinical features:

Patients are generally young female. Finding include:

1. Constitutional symptoms like fatigue, fever and weight loss.

2. Arthritis: usually migratory and asymmetric, involving mostly small joints of the hands.

3. Skin Involvement: It includes;

• Malar rash ( butterfly rash over the cheeks and nose). 
• Discoid rash (scaling papules that can leave residual scaring), 
• Alopecia, 
• Painless oral ulcers,
• Raynaud's phenomenon,
• Photosensitive rash. 

4. Renal failure.

5. Pleuritis, pleural effusion, interstitial lung disease, pulmonary hypertension. 

6. Pericarditis and pericardial effusion.

7. Psychological symptoms like anxiety, depression and psychosis.

8. Seizures, headache and neuropathies. 

9. Hematological abnormalities thah include thrombocytopenia, hemolytic anemia, leukopenia.

10.Peritonitis. 

Diagnosis:

The diagnostic criteria for SLE can be memorized by the mnemonic DOPAMINE RASH

• Discoid rash
• Oral ulcer
• Photosensitive rash
• Arthritis
• Malar rash
• Immunologic criteria (+ anti ds-DNA or + anti- Sm )
• NEurologic or psychiatric symptoms.
• Renal disease.
• ANA +
• Serositis (pleural, peritoneal, or pericardial )
• Hematological disorders ( Thrombocytopenia, anemia, leukopenia )

ANA is highly sensitive but non-specific, whereas anti-ds DNA and anti-smooth muscle antibodies are highly specific. 

Treatment: 

1. Arthritis and mild serositis are treated with NSAIDs. 

2.Hydroxychloroquine is used for rashes and for arthritis that is unresponsive to NSAIDs. 

3. Steroids and immunosuppressants are used in the presence of significant organ involvement. 

4. Patients undergoing active SLE flare ups are treated with steroids, which are tapered when remission is induced. .

5. Patients with antiphospholipid antibody syndrome need life long anticoagulation with warfarin.