A 71-year-old woman with no history of interest who after 6 months of dermatitis on her face, trunk and extremities was diagnosed in July 2013 by skin biopsy of DM with cutaneous involvement, without muscle weakness.
Muscle enzymes CK and aldolase are normal.
Positive ANA antibodies (1:320) and Mi-1:Mi-2 (1:320) were detected with negative anti-P155/140 antibodies.
Tumor markers and a complete CT scan showed no evidence of malignancy.
Acute phase reactants are elevated, CRP 44.2 mg/L [0-5] and VSG 73 mm/h [0-19].
It is treated with prednisone at a dose of 30 mg/24 h in various descending regimens for 4 months.
During this period, progressively begins with myalgias and muscle weakness that forces you to be bedridden, with fever, dysphagia and loss of 10 kg of weight.
In December 2013, the patient was admitted with suspected MD and myopathy.
Electromyogram (EMG), magnetic resonance imaging and muscle biopsy were performed to confirm inflammatory myopathy in the proximal muscles of the extremities.
Pathogram confirmed significant esophageal motor impairment and delayed gastric emptying.
It is diagnosed with DM with muscle and digestive involvement, as well as cutaneous involvement.
The pulmonary study and the echocardiogram are normal.
Corticosteroid therapy was maintained in the first 8 days prior to admission.
Kinapride is prescribed as a prokinetic agent.
On the ninth day, after confirming myopathy, azathioprine 50 mg/24h was initiated.
Intravenous (IV) pulses of methylprednisolone were added for 3 days, followed by prednisone 30 mg/24h for 2 weeks (after a descending regimen).
On the seventh day of starting azathioprine, AST/ AST 160 U/L [2 - 35] and ALT/ALT 313 U/L [2 - 35] were detected, with normal previous levels.
It was decided to suspend azathioprine due to hepatotoxicity and the pharmacist reported an adverse reaction to the Centre for suspicion of vigilance.
Mycophenolate 500 mg/24h was initiated for which the patient signed an informed consent form and documentation for the use of medications in special situations was obtained.
The dose is increased to 1 g/12h.
1.
After 6 weeks of treatment, the patient reported improvement in muscle strength, increased physical activity, significant reduction in dysphagia and dysphagia, with normalization of acute phase reactants and transdolase, continuing normal with CK and cholinergic reactions.
It was decided to suspend cinitapride and continue with a descending prednisone regimen.
After 9 weeks of therapy, the patient complained of hair loss and paresthesia in the lower limbs, which were described as common adverse reactions in the Summary of Product Characteristics for mycophenolate.
At week 12, the prednisone dose was reduced from 10 mg to 7.5 mg/24h.
At week 14, the patient described a 10 cm pruritic erythematous plaque similar to those presented at the onset of the disease.
In analytical analysis, the only change observed was the increase in CRP to 29.2 mg/L. Alopecia disappears and paresthesias disappears and it was decided to return to 10 mg of prednisone every 24 hours.
At week 16, CRP returned to 4.1 mg/L and the skin condition disappeared and returned to the prednisone dose of 7.5 mg/24h.
In the control at week 20, CRP remains in range and there are no signs of dermatitis, considering that prednisone therapy 7.5 mg/24h plus 1 g/12h of mycophenolate is safe and effective to control the disease.
