CASE REPORT
A 51-year-old female patient with a 10-year history of psoriatic
arthritis developed paroxysmal cough and cough with white, sticky sputum
after a cold 5 months ago, accompanied by chills, general malaise, chest
tightness, shortness of breath, back pain, and hoarseness after
exercise. She did not improve after cold treatment. Two months later,
shortness of breath after activity and speech worsened, and there was
scattered erythema on the neck, some of which merged into a piece, rough
skin on the radial side of the right index finger, the distal end of the
hands, and white skin after cold; a chest CT was performed at an
external hospital. Interstitial infection in the right lobe, lingual
segment of the upper lobe of the left lung, and lower lobes of both
lungs was observed, and electronic bronchoscopy showed no abnormalities.
Pulmonary function tests revealed moderate restrictive ventilatory
dysfunction, small airway dysfunction, and moderate diffusion
impairment. Electromyography revealed a trend toward myogenic injury in
the proximal muscles of the extremities. Bilateral interstitial
pneumonia and connective tissue disease were also considered. To combat
the infection, methylprednisolone 40 mg once daily and pirfenidone 200
mg three times daily were used for treatment, and the cough and sputum
improved, with no improvement in shortness of breath after exercise,
without further deterioration. After four months of activity, chest
tightness, shortness of breath, cough, and white sputum appeared. She
went to the Department of Respiratory Medicine, West China Hospital,
Sichuan University, for examination of anti-Mi-2α, anti-MDA5, and
anti-PM Scl antibodies (+-). CT scan of the Chest computed tomography
revealed vitreous opacities, patchy shadows, and grid-like shadows in
both lung membranes, mostly due to interstitial inflammation in both
lungs. Biopsy of the left deltoid muscle dermatomyositis skeletal
myopathy could not be excluded, followed by methylprednisolone
modulation of immunity and no significant relief of symptoms after
anti-infection therapy. We considered that our department should check
for positive anti-MDA5 antibodies to diagnose DM and to check the finger
pulse. Oxygen 89% (no oxygen inhalation), dry and wet rales could be
heard, a small amount scattered in the lower lungs, veclro rales could
be heard in the bases of both lungs, chest CT showed chronic
interstitial changes in both lungs; mixed type in the lower lobes of
both lungs Possibility of infection, fungus, procalcitonin(Figure 1) , C-reactive protein, erythrocyte sedimentation rate,
muscle enzyme spectrum, electrolytes, thyroid function, tumor markers
were normal, excluding other autoimmune diseases and tumors, sputum
culture was Lewy persistent bacillus infection, antibiotic
anti-infective treatment and methylprednisolone 40 mg qd (reduced by 4
mg per week), cyclosporine 25 mg bid, pirfenidone 200 mg tid treatment,
cough, sputum, chest tightness, shortness of breath, muscle pain
significantly improved, no skin damage.
After 12 weeks of treatment, the patient visited Peking Union Medical
College Hospital and was found to be anti-MDA5 positive. The patient was
treated with methylprednisolone (12 mg qd), tacrolimus (2 mg qd),
cyclophosphamide (50 mg qd), and pirfenidone (200 mg). No rash was
observed after treatment. Muscle pain was present, but chest tightness,
shortness of breath, cough, and sputum production were observed.
After 36 weeks of treatment, considering the toxic side effects of
cyclophosphamide, after excluding infection, tumor, tuberculosis, and
hepatitis, we adjusted the treatment plan to methylprednisolone 6 mg qd,
tacrolimus 2 mg qd, tofacitinib 5 mg bid, and pirfenidone 200 mg tid.
Chest tightness and shortness of breath, cough, and expectoration
continued to be relieved, and hormone levels were successfully reduced
after 52 weeks of treatment. Tacrolimus Capsules 1 mg qd remained in
remission and underwent multiple follow-ups; the anti-MDA5 antibody was
positive many times using the Cytometric Bead Array (CBA) detection
method.
At the 132 weeks of follow-up, we comprehensively evaluated the
patient’s condition. Pulse oxygen was 95% (without oxygenation), and
pulmonary function was normal. After repeated checks, the anti-MDA5
antibody was negative, and the symptoms continued to be relieved. First,
we continued to administer tacrolimus division 1 mg qd, tofacitinib 5 mg
bid, and pirfenidone 200 mg qd maintenance therapy (Figure 2) .
It is worth noting that the patient’s muscle enzyme, immunoglobulin,
C-reactive protein, and erythrocyte sedimentation rates were normal from
the onset to the 132-week follow-up(Figure 3) .