Question

A 26-year-old woman presented with painful, stiff knees of 4 weeks duration. She had a 6-year history of Raynaud's phenomenon. On examination, she had bilateral effusions in both knee joints, but all other joints were normal. She had no skin lesions, muscle tenderness, proteinuria or fever. The results of relevant investigations are shown in Table C10.1. On the basis of these, a diagnosis of__________________?????was made and the patient treated with aspirin for her painful knees. She improved over 4 weeks and then remained symptom-free for 5 years. During this time, her antinuclear antibody remained positive at 1/80, her DNA-binding activity varied from 40 to 80%, and her C3 and C4 levels were occasionally low. Later, she developed a bilateral, blotchy rash on her hands and thighs, consistent with active vasculitis. Her Raynaud's phenomenon concurrently became much worse. Following treatment with prednisolone, the skin manifestations gradually disappeared and the steroids were tailed off.

This patient presented with arthritis and Raynaud's phenomenon. She is unusual in that the arthritis of_____________???? usually involves small joints, but it is important to note that she remained perfectly well without treatment for 5 years, despite persistently abnormal serology.

C-reactive protein	8mg/l (normal)
Rheumatoid factor	Negative
Antinuclear antibody	Positive (titre 1/80; IgG class)
dsDNA-binding activity	80% (NR 0-30% binding)
Antibodies to extractable nuclear antigens	Negative
Serum complement levels
C3	0.35g/l (NR 0.65-1.30)
C4	0.05g/l (NR 0.20-0.50)
Serum immunoglobulins
IgG	22.0g/l (NR 7.2-19.0)
IgA	3.8g/l (NR 2.0-5.0)
IgM	1.2g/l (NR 0.5-2.0)
Biopsy of normal, sun-exposed skin (lupus band test)	Granular deposits of IgG and complement at dermo-epidermal junction

Answer 1

Answer : The patient suffers from SLE ( Systemic Lupus Erythematosus).Both the blanks should be filled with SLE.

Explaination : According to the SLE diagnosis guidelines laid by the American College of Rheumatology, atleast four of the 11 criterias should be present to be able to diagnose SLE.

The four positive criteria in the given case are as follows :

1. Musculoskeletal involvement

2. Positive antinuclear antibody (compulsory criteria)

3. Positive SLE specific antibodies ie; dsDNA-binding activity

4. Low levels of C3, C4 proteins

In addition to the above four positive criteria the patient in the above case has Granular deposits of IgG and complement at dermo-epidermal junction found on Lupus band test.

Hence we can come to a conclusion that this is a case of SLE.