Clujul Medical - Iuliu Haţieganu

Clujul Medical 2006 vol. LXXX - nr. 1
Case report
We report the case of a 53 year old female admitted to the Hospital Medicala V
for drowsiness, fatigue, malaise, dizziness, diaphoresis, progressive shortness of breath
with effort but also at rest, reduced exercise capacity, anorexia, abdominal pains,
nausea, severe weight loss (approximately 50 kg in 2 months), low-grade fever,
transient bilateral knee pains and swelling, progressive stiffening of both hands with
functional impairment and diffuse bilateral lumbar pain. The physical examination
revealed an impaired general condition, altered nutritional status, characteristic facial
expression (suggestive for systemic sclerosis), erythematous lesions on the skin
(covered by squamae), discoid rash located on the palms and digits of both hands
(probably livedo reticularis of vasculitic nature) associated with areas of necrotic
lesions, epidermal atrophy, induration, loss of mobility and retraction with
immobilization of the fingers in a semiflexed position. Other findings included: interosseous
atrophy, mild cubital deviation and bilateral Raynaud phenomena of the
extremities. Raised red scaling plaques with central atrophy on the extremities, some of
them having resolved causing hyper-pigmentation, atrophy and scarring, friable nails,
brittle hair and patchy alopecia were also noticed. Examining the oral cavity we found
multiple painful ulcerations on the soft palate. Polyadenopathy, with firm, mobile,
untender cervical, axillary and inguinal lymph nodes was identified. The assessment of
the cardiovascular system revealed an increased area of cardiac dullness, tachycardia (
HR= 120/min), hypertension (150/100 mm Hg), faint rhythmic heart sounds and
pericardial friction rub. On examining the abdomen we found a significant enlarged
liver (5 cm below the right costal margin), mild epigastric tenderness.
The laboratory results indicated anaemia (Hb = 11,3g/dl, Ht = 34%, H =
3,82mil/mm3), inflammatory syndrome ( ESR = 80/110 mm, CRP = 3,7 UI/ml), hepatic
dysfunction (ASAT = 198 U/l, ALAT = 78 U/l, FA = 372 U/l, GGT = 181 U/l, Bilirubin
- normal range), renal dysfunction (24-hour urine protein = 0,85g/24 h, blood urea and
creatinine - normal range) and also raised the suspicion of rhabdomyolysis (Total CPK
= 428 U/l). The microbiological exams revealed urinary tract infection (Enterobacter >
100.000 UFC/ml ), oral candidosis and negative blood culture.
The chest X-ray revealed a globally enlarged cardiac silhouette with a pleural
collection in the left costophrenic sinus. The admission electrocardiogram showed a low
QRS voltage. The echocardiography described medium/large pericardial effusion
(exudative pericarditis) with tendency for diastolic collapse of the right ventricle and
right atrium. The abdominal ultrasound showed liver enlargement, with diffuse
increased hepatic ecogenicity and ascites in moderate quantity. The Barium X-ray exam
doccumented gastric dysmotility and hypotonia with mildly diminished peristaltic
movements and stasis.The X-ray examination of the hands and knees revealed lesions of
osteoarthritis in the distal interphalangeal joints and knees.
The immunological profile revealed the presence of ANA ( antinuclear
antibodies 1/1280 --> speckled pattern) and also of Anti-Sm (Smith antigen) and AntinRNP
(nuclear ribonucleoproteins). The anti DNA antibodies (double stranded DNA),
the ANCA (anti-neutrophil cytoplasm antibodies), the SMA (smooth muscle antibody),
the AMA (antimitochondrial antibody) were all negative. The Ig G anti-cardiolipin was
4 U, RF (Rheumatoid Factor) = 32 UI/ml, CIC (circulatory immune complexes) = 460 x
10-3, ASLO (anti-streptolysin O) – negative, Cryoglobulin – positive, VDRL –
negative, C3 = 21 mg%, C4 = 7 mg%, CRP (C-reactive protein) = 3,7 mg/dl, Ig G =
1922 U/ml, Ig M = 200 U/ml, Ig A = 122 U/ml.
Both the skin biopsy (from the finger lesions) and the deltoid muscle biopsy
did not reveal typical aspects of vasculitis or characteristic lesions of myositis, in spite
of the suggestive clinical appearance, but they were carried out after 2 weeks of
corticotherapy. The axillary lymph node biopsy was not relevant.
The data obtained did not permit us to include this case in a typical, well-
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