A 40-year-old male patient, a lawyer from Mexico City, with no relevant chronic-degenerative background, presented with dry cough and moderate dyspnea for the last 8 months, which had gradually worsened. By this time, he had already been examined a couple of times and prescribed inhaled muscarinic bronchodilators, with partial improvement on the symptoms. Nonetheless, skin peeling on distal phalanges on both hands has emerged 15 days before his visit to the hospital, with no apparent trigger (Figure 1).
Evolving over time with functional class deterioration, the patient presents paroxysmal coughing and hemoptysis; hence, his hospital admission with clinical evidence of hypoxemia and moderate anaemia (hemoglobin 8.2 gr/dl).
Once hospitalized, the physical exam revealed jugular vein distention (JVD), preserved muscle strength, and subcrepitant rales in the subscapular region. Consequently, a pneumopathy research was initiated through a transthoracic echocardiogram (TTE), which showed a left ventricular concentric hypertrophy, preserved ejection fraction, and an increased pulmonary artery systolic pressure suggesting a moderate pulmonary hypertension. However, the patient persisted with respiratory deterioration, requiring invasive mechanic ventilation and further admission to the Intensive Care Unit (ICU), where he was initiated with an empirical antibiotic treatment. After 48 hours of showing a poor response to the antibiotic treatment, the staff decided to perform a high-resolution computed tomography, reporting a matching pattern of a nonspecific interstitial pneumonia (NSIP) (Figure 2).
Based on these findings, a bronchoalveolar lavage was performed showing the presence of loaded hemosiderin macrophages, confirming a diffuse alveolar hemorrhage; hence, a 1000 mg pulse IV methylprednisolone and cyclophosphamide therapy was established for 3 days.
A screening research for pauci-immune vasculitis was performed; nevertheless, organ damage and anti-neutrophil cytoplasmic antibodies (ANCAs) were not identified. Additionally, other causes of immune complex-mediated vasculitis were studied, but hepatitis B/C virus, tuberculosis, and HIV values were all negative and complement levels were normal. The sole finding that suggested that the patient was suffering from an autoimmune disease and was a strongly positive antinuclear antibodies ratio (1 : 1280), with cytoplasmic and ribosomal pattern, which implied to discard systemic lupus erythematosus. However, there was not enough evidence to justify a renal function deterioration, proteinuria, haematuria, or pyuria.
Although the patient did not meet the classification criteria for systemic lupus erythematous, the lookup for specific antibodies for myositis was conducted, highlighting an anti-PL-12 and anti-Ro-52 strongly positive result. Accordingly, subclinical myositis was confirmed through electromyography; results showed a recruitment pattern, even when the patient had never presented muscle enzyme elevation or decreased muscle strength in a significant manner. Conclusive diagnosis included incomplete antisynthetase syndrome and alveolar hemorrhage.
Once out of the intensive care unit, a prednisone 50 mg/day and methotrexate 15 mg/wk was followed for 3 months until the dyspnea and hemoptysis disappeared. Confirmed negative tests on neoplastic disease assured the patient's amelioration. One year later, the patient showed a considerable improvement of his respiratory function parameters. As a regular follow-up treatment, the patient is on low doses of methotrexate, practically asymptomatic.