Introduction
The idiopathic inflammatory myopathies (IIMs) comprise
a heterogeneous group of diseases of unknown etiology
characterized by chronic inflammation of the skeletal muscles.
IIMs are rare in subjects of reproductive age and very
few cases of pregnancy in women affected by IIMs have
been reported in the literature. Furthermore, these have
generally been associated with a poor pregnancy outcome
and relapses of disease activity, suggesting a negative effect
of pregnancy on disease activity and vice versa (1–7).
Here we describe the case of a woman with classic dermatomyositis
(DM) that developed at the beginning of her
pregnancy. She was treated with intravenous immunoglobulin
therapy (IVIG) and corticosteroids and was delivered
of a healthy 3,180-gm boy at 35 weeks’ gestation.
Case report
A 32-year-old woman affected by dermatomyositis was
referred to the Rheumatology Unit of Pisa University in
June 2002, when she was 17 weeks pregnant. Disease onset
dated to March 2002 with the appearance of diffuse myalgias,
increasing proximal muscle weakness, and skin rash;
blood tests at the time showed a marked increase in
transaminases and creatine phosphokinase (CPK; 3,560
IU/liter, normal values 180 IU/liter). During the same
timeframe, she underwent a pregnancy test, which proved
positive. The patient was admitted to a local hospital
where the diagnosis of DM was made on the basis of the
findings of a heliotrope rash and Gottron’s papules, an
increase in muscle enzyme plasma levels, and a positive
electromyogram (8). Moderate-dose steroid therapy (methylprednisolone
40 mg/day) was instituted, with a prompt
improvement of the serologic markers (CPK 1,836 IU/liter).
After this initial positive response in May 2002, however,
the patient presented with a progressive increase in muscle
weakness, a worsening of the cutaneous manifestations,
and a further increase in CPK levels (up to 3,650
IU/liter). Her steroid dosage was increased to 60 mg/day
methylprednisolone and she was transferred to the Rheumatology
Unit of the University of Pisa.
Examination on admission revealed the presence of a
heliotrope rash, Gottron’s papules, and severe proximal
muscle weakness in the shoulder and hip girdles. There
was no peripheral edema, and her blood pressure values
and chest and cardiac examinations were normal. The
results of her laboratory tests were as follows: CPK 1,163
IU/liter; aspartate aminotransferase 141 U/liter (normal
values 45 U/liter), alanine aminotransferase 91 U/liter
(normal values 45 U/liter), normal renal function, no
proteinuria, and a negative Coombs test. She was positive
for antinuclear antibodies by indirect immunofluorescence
(1/160 with a diffuse pattern), whereas assays for
anti-Ro/SSA, anti-La/SSB, anti-Sm, anti-RNP, and anti-Jo1
antibodies by CIE were negative as were anticardiolipin
antibodies and lupus anticoagulant. Capillaroscopy
showed the presence of diffuse abnormalities with a polydermatomyositis
pattern.
The obstetric evaluation was unr...