ABSTRACT
Idiopathic, nonspecific interstitial pneumonia (NSIP) is most often associated with various clinical disorders, including connective tissue diseases (CTDs) and chronic hypersensitivity pneumonitis (cHP). Emerging evidence also suggests that "idiopathic" NSIP may be the lung manifestation of undifferentiated CTD (UCTD). However, whether or not NSIP outcome is influenced by the underlying cause remains uncertain. This retrospective study included 127 biopsy-proven NSIP patients (65 women, mean ± sd age 55 ± 12 years). Survivals were estimated using a Kaplan-Meier curve and compared using the log-rank test. Multivariate analyses were based on a Cox model. 15 (11.8%) patients had cHP, 29 (22.8%) had CTD, 32 (25.2%) satisfied the Kinder criteria for UCTD and 51 (40.1%) had idiopathic NSIP. At the end of follow-up (mean ± sd 64 ± 54 months), a difference in survival was observed between aetiological groups (p=0.002). Survival was better for UCTD than for idiopathic NSIP (p=0.020) and similar to that observed for CTD. cHP survival tended to be poorer than that of idiopathic NSIP (p=0.087) and was an independent predictor of mortality (hazard ratio 2.17, 95% CI 1.05-4.47; p=0.035). NSIP outcome is influenced by its cause. cHP exhibits the highest mortality. UCTD does not differ from CTD supporting the concept of autoimmune NSIP, with a prognosis that is better than that of idiopathic NSIP.
Subject(s)
Alveolitis, Extrinsic Allergic , Connective Tissue Diseases , Idiopathic Interstitial Pneumonias , Lung , Adult , Aged , Alveolitis, Extrinsic Allergic/complications , Alveolitis, Extrinsic Allergic/diagnosis , Alveolitis, Extrinsic Allergic/immunology , Autoantibodies/blood , Biopsy , Cause of Death , Connective Tissue Diseases/complications , Connective Tissue Diseases/diagnosis , Connective Tissue Diseases/immunology , Diagnosis, Differential , Female , France/epidemiology , Humans , Idiopathic Interstitial Pneumonias/diagnosis , Idiopathic Interstitial Pneumonias/etiology , Idiopathic Interstitial Pneumonias/immunology , Idiopathic Interstitial Pneumonias/mortality , Kaplan-Meier Estimate , Lung/diagnostic imaging , Lung/pathology , Male , Middle Aged , Outcome Assessment, Health Care , Prognosis , Proportional Hazards Models , Respiratory Function Tests/methods , Retrospective Studies , Tomography, X-Ray Computed/methodsABSTRACT
History A 55-year-old man presented with chronic epigastric pain lasting for about 1 year and without fever or vomiting. The abdomen was soft and tender at physical examination. Laboratory tests revealed unremarkable liver function, normal hemoglobin level, and normal amylase level. White blood cell count was normal, and there was no inflammatory syndrome. The patient's medical history included pancreatic gastrinoma resected by means of left pancreatectomy 31 years before, hyperparathyroidism treated with subtotal parathyroidectomy 24 years before, and a slowly growing lung mass known for 9 years. Esophagogastroduodenoscopy was performed because of a suspected gastroduodenal ulcer. The results showed numerous small (<10 mm) gastric and duodenal ulcers and multiple 10-15-mm polypoid gastric masses. Contrast material-enhanced dual-phase multidetector row computed tomography (CT) of the chest and abdomen was performed with a 64-section CT scanner (LightSpeed VCT; GE Healthcare, Milwaukee, Wis). Technical parameters for CT were as follows: pitch, 0.98; section thickness and reconstruction interval, 1.25 mm; 120 kVp; and variable milliamperage determined by x-, y-, and z-axis dose modulation. After an unenhanced abdominal scan, iobitridol, a nonionic iodinated contrast agent containing 350 mg of iodine per milliliter (Xenetix 350; Guerbet, Aulnay-sousbois, France), was administered intravenously through a 16-18-gauge catheter. A 120-mL dose of the contrast agent was injected via an antecubital vein at a rate of 4 mL/sec. No oral contrast medium was administered. After preliminary unenhanced abdominal scanning, arterial and portal venous phase acquisitions were obtained 45 and 80 seconds after initiation of contrast medium injection.
