Objective
The aim of this study was to determine the association of demographic parameters, clinical manifestations, disease activity, and pharmacologic therapy with disease damage in a group of Puerto Ricans with primary Sjögren syndrome (pSS).
Methods
A cross-sectional study was conducted in 100 Hispanics of Puerto Rico with pSS. Patients were 21 years or older and fulfilled the 2012 American College of Rheumatology classification criteria for pSS. Demographic factors, lifestyle behaviors, extraglandular manifestations, serologic tests, comorbidities, pharmacologic therapy, disease activity (per European League Against Rheumatism Sjögren Syndrome Disease Activity Index), and disease damage (per Sjögren Syndrome Disease Damage Index [SSDDI]) were assessed. Patients with disease damage (SSDDI ≥1) and without damage (SSDDI = 0) were compared using bivariate analysis and multivariate regression analysis adjusted for age, sex, and disease duration.
Results
The mean age of patients was 52.8 years; 94% were women. The mean disease duration was 5.9 years. Thirty-nine patients had disease damage. Disease damage was mainly attributed to pulmonary fibrosis and peripheral neuropathy. In the bivariate analysis, disease damage was associated with low C3 and C4, coronary artery disease, infections, and higher activity index and was more frequently treated corticosteroids and azathioprine. In the multivariate analysis, low C3, disease activity, and corticosteroid exposure retained significance.
Conclusions
In this population of Puerto Ricans with pSS, C3 and C4 hypocomplementemia, coronary artery disease, infections, and exposure to corticosteroids and azathioprine were associated with damage accrual. Clinicians should be aware of these factors to identify those who may require close follow-up and early therapeutic intervention.
DESCRIPTIONA 38-year-old woman with systemic lupus erythematosus (SLE) manifested by tiredness, polyarthritis, lymphopaenia, elevated antidouble-stranded DNA antibodies, and hypocomplementaemia was initiated on azathioprine and hydroxychloroquine. After 2 weeks of treatment, she developed extensive erythematous raised skin patches, facial oedema, and desquamation of nasal and oral mucosa. Both drugs were discontinued and she improved within 4-7 days. Since hydroxychloroquine is associated with more hypersensitivity reactions than azathioprine, the latter was reintroduced, but 2 days later, the patient developed severe recurrent skin lesions ( figure 1A-C). Skin biopsy showed band-like lymphocytic infiltrate with focal vacuolar interface
Nocardia is an ubiquitous microorganism found in soil, organic matter, and water. It is particularly common in immunocompromised patients. A 51-year-old man, nonsmoker with medical history of hypertension and chronic alcoholism, presented with a 2-week history of a right upper quadrant pain, right-side chest pain, general malaise, fatigue, anorexia, 20-lb weight loss, night sweats, unquantified fever, and visual/auditory hallucinations. An abdominal and chest computed tomography reported a right pleuraYbased mass that eroded into the chest wall. The patient was admitted with diagnosis of suspected primary lung malignancy with a postobstructive pneumonic infection. Intravenous antibiotic was started with vancomycin and piperacillin/tazobactam. Computed tomographyYguided needle biopsy was done and pathology reported a gram-positive filamentous branching bacteria, weakly acid-fast positive, consistent with a Nocardia infection. Trimethoprim/sulfamethoxazole therapy, together with pleural effusion management, ensured further resolution of this invasive pleuropulmonary infection. FIGURE 3. Pulmonary nocardiosis pathology and stain with Papanicolaou at original magnification Â40.FIGURE 4. Pulmonary nocardiosis pathology and stain with Papanicolaou at original magnification Â20.FIGURE 5. Chest CT scan before (left) and after (right) treatment showing resolution of pleuraparenchymal changes.
Campos-Santiago et al
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