Clinical and Functional Outcomes Associated with Quality of Life in Patients with Lymphangioleiomyomatosis: A Cross-Sectional Study.

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare (twenty-one per million female inhabitants) neoplastic cystic lung disease that impairs health-related quality of life (HRQoL). However, the factors associated with impaired quality of life in patients with LAM are poorly understood. OBJECTIVE: To assess the clinical, psychosocial, and functional characteristics associated with impaired quality of life in patients with LAM. METHODS: This was a cross-sectional study performed on two nonconsecutive days. HRQoL (SF-36 and CRQ), lung function tests, anxiety and depression symptoms (HADS), maximal (CPET and ISWT), and submaximal exercise capacity (6MWT) were assessed. Linear associations among outcomes were assessed using Pearson's correlation and multivariate tests. RESULTS: Forty-five women with LAM (46 ± 10.years; FEV1,74%pred) were evaluated. The lowest SF-36 scores were observed for general health and vitality and the highest for the physical and social domains. The lowest CRQ scores were observed for dyspnea and fatigue, and the highest were for the emotional function and self-control domains. Sixteen (35%) women had anxiety, and 8 (17%) had depression symptoms. Most of the SF-36 and CRQ domains were associated with anxiety and depression symptoms (from r = 0.4 to r = 0.7; p < 0.05) and exercise capacity (from r = 0.3 to r = 0.5; p < 0.05). Lung function parameters were weakly or not associated with quality of life domains. After multiple linear regression, HRQoL was independently associated with depression symptoms and physical capacity but not with lung function. CONCLUSION: Our results show that aerobic capacity and depression symptoms are the main factors, rather than lung function, related to quality of life in patients with LAM.

Evaluation of maximal exercise capacity through the incremental shuttle walking test in lymphangioleiomyomatosis.

BACKGROUND: The cardiopulmonary exercise test (CPET) is the gold standard for assessing aerobic fitness; however, it is expensive, not widely available, and requires specialized equipment and staff. The incremental shuttle walking test (ISWT) is an exercise field test used to evaluate exercise capacity and may be an alternative to CPET in patients with lymphangioleiomyomatosis (LAM). OBJECTIVE: To investigate whether the ISWT can be used to assess maximal aerobic capacity in patients with LAM. METHODS: Forty-five women were evaluated on two days, and they randomly performed the CPET and ISWT. The maximum oxygen uptake (peak VO2) was evaluated using gas analyzers in both tests. The carbon dioxide production (VCO2), respiratory exchange ratio (RER), and heart rate (HR) were compared during peak exercise. Pearson's correlation and Bland-Altman assessed the association and agreement, respectively. The intraclass correlation coefficient (ICC) was used to assess the reliability of the data. RESULTS: All patients (46.1 ± 10.2 years) presented similar peak VO2, RER, and peak HR during the CPET and ISWT (15.6 ± 4.6 vs. 15.7 ± 4.4 ml·kg-1·min-1; 1.15±0.09 vs. 1.17±0.12; and 142.2 ± 18.6 vs. 141.5 ± 22.2 bpm, respectively; p>0.05). A good linear correlation (r = 0.79; p<0.001) and ICC (0.86; 95%CI 0.74-0.93) were observed between the peak VO2 in both tests. Predictive peak VO2 equations based on the ISWT performance are also presented. CONCLUSION: Our results suggest that the ISWT can be used to assess maximal exercise performance in patients with LAM, and it is a valuable option to be used as an alternative to the CPET and predict maximal exercise capacity.

Isolated Cystic Lung Disease: An Algorithmic Approach to Distinguishing Birt-Hogg-Dubé Syndrome, Lymphangioleiomyomatosis, and Lymphocytic Interstitial Pneumonia.

OBJECTIVE. Birt-Hogg-Dubé (BHD) syndrome, lymphangioleiomyomatosis (LAM), and lymphocytic interstitial pneumonia (LIP) frequently present as isolated cystic lung disease and can be challenging to distinguish. If imaging findings are otherwise unremarkable, the radiologist is unaided by ancillary CT findings in narrowing the diagnosis. We hypothesized that the distribution and morphologic features of lung cysts could be used to differentiate BHD syndrome, LAM, and LIP. Therefore, the purpose of this study was to characterize the CT appearances of these conditions and create a practical CT-based algorithm to differentiate among them. MATERIALS AND METHODS. The study was a retrospective review of the CT images of 16 patients with BHD syndrome, 17 patients with LAM, and 14 patients with LIP. On the basis of the data collected, a CT-based algorithm was created, and the CT images were reviewed again. RESULTS. Lower lung-predominant cysts were significantly more likely to be found in patients with BHD syndrome (100% of patients) or LIP (71-93% of patients) than in patients with LAM (6-12% of patients), who were more likely to have diffuse cysts. Compared with patients with LIP or LAM, patients with BHD syndrome were significantly more likely to have elliptical (floppy) paramediastinal cysts (88-94% of patients with BHD syndrome, 36-43% of patients with LIP, and 6-12% of patients with LAM) or a disproportionate number of paramediastinal cysts (69-88% of patients with BHD syndrome, 0-14% of patients with LIP, and 0-6% of patients with LAM). Our algorithm enabled differentiation of BHD syndrome, LAM, and LIP with a high level of accuracy and high interreader agreement (κ = 0.809). CONCLUSION. Radiologists can use the proposed CT-based algorithm to prospectively and confidently suggest one of these disorders as the favored diagnosis. Of importance, this will allow diagnosing the disorder early and accurately, screening for comorbidities, and prevention of potential complications.

Síndrome de Sjögren primario y enfermedad quística pulmonar: reporte de un caso / Primary Sjögren syndrome and pulmonary cysts disease: case report

El síndrome de Sjögren (SS) es una enfermedad inflamatoria, autoinmunitaria crónica de etiología desconocida, caracterizada por infiltración del tejido glandular y extraglandular por linfocitos y células plasmáticas. Presenta manifestaciones glandulares (xeroftalmia y xerostomía) y extraglandulares (50%); v.gr.: pulmonares (11%) ­las más comunes son la enfermedad intersticial linfocítica y la enfermedad quística pulmonar (25%)­; las manifestaciones extraglandulares preceden, muchas veces, a las manifestaciones glandulares; por este motivo su diagnóstico se confunde o se retrasa. Caso clínico: paciente de sexo femenino, 71 años de edad, con antecedentes de hipertensión arterial e hipotiroidismo. Hospitalizada por astenia, tos seca y disnea de medianos esfuerzos. Revisión de aparatos y sistemas: xeroftalmia y xerostomía. Exámenes complementarios: Rx tórax: opacidades difusas redondeadas en ambos campos pulmonares; tomografía simple de tórax: múltiples lesiones quísticas predominantes en los lóbulos inferiores y calcificaciones nodulares. Se amplían los estudios para determinar la etiología: exámenes inmunoquímicos: anti SSA/Ro 200 U/ml (< 20 UE/ ml), anti SSB/La 84,4U/ml (< 20 UE/ml), anticuerpos antinucleares 1/160 (≤ 1/40), factor reumatoideo 49,9UI/ml (< 20 UI/ml). Se sospecha síndrome de Sjogren y realiza sialografía de parótidas que evidencia obstrucción y procesos inflamatorios de los conductos parotídeos. Biopsia de labio inferior: mucosa revestida por epitelio escamoso, edema intercelular y exocitosis linfocitaria. El estroma contiene infiltrado linfomononuclear disperso característico del SS. Una vez confirmado el diagnóstico, se trata con corticoides y tratamiento específico del ojo y boca seca.

Context: Sjögren's syndrome (SS) is an inflammatory, chronic autoimmune disease of unknown etiology, which is characterized by infiltration of glandular and extraglandular tissue by lymphocytes and plasma cells. It presents glandu-lar manifestations (xerophthalmia and xerostomia), as extraglandular (50%). Within these, there are pulmonary manifestations (11%), the most common manifestation being lymphocytic interstitial disease followed by cystic lung dis-ease (25%). Its manifestations often precede the glandular manifestations so that the diagnosis of it is confused or delayed. Case presentation: A 71-year-old female patient with a history of high blood pressure and hypothyroidism. Hospitalized for presenting asthenia, dry cough and dyspnea to the medium efforts. In the review of all systems, she referred xerophthalmia and xerostomia. The following exams were carried out: Rx thor-ax: rounded diffuse opacities in both pulmonary fields, is complemented with simple chest Tomography: Multiple pre-dominant cystic lesions in lower lobes and nodular calcifications. It was decided to expand the studies to determine etiology and see if it is related to its xerophthalmia and xerostomia characteris-tic of SS. Immunochemical tests were performed. The results: anti-SSA / Ro 200 U / mL (< 20 EU / mL); anti SSB / 84.4U / mL (< 20 EU / mL); antinuclear antibodies with fine granular pattern 1/160 (≤ 1/40) Rheumatoid factor 49.9UI / mL (< 20 IU / mL). With the suspicion of Sjogren's syndrome, parotid sialog-raphy is performed, obstruction is evidenced and chronic inflammatory pro-cesses of parotid ducts. Lower lip biopsy: Mucosa lined by squamous epitheli-um with intercellular edema and exocytosis of small lymphocytes. The stroma contains mild scattered lymphomononuclear infiltrate characteristic of SS. Con-firmed diagnosis of SS managed with corticosteroids and specific treatment of eye and dry mouth.

Diffuse cystic lung disease due to pulmonary metastasis of colorectal carcinoma.

The diffuse cystic lung diseases (DCLDs) are a pathophysiologically heterogeneous processes characterized by the presence of multiple thin-walled, air-filled spaces within the pulmonary parenchyma. The most common causes of DCLD are lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH). DCLD develops rarely as a result of malignancy, typically secondary to metastases from peripheral sarcomas and mesenchymal tumors. DCLD have also been reported in a variety of other metastatic disease such as adenocarcinoma. Our case describes a patient with DCLD as a result of metastatic colorectal adenocarcinoma.

Síndrome de Sjögren primario con neumonía intersticial linfocítica y enfermedad quística pulmonar / Primary Sjögren's syndrome with lymphocitic interstitial pneumonia and cystic pulmonary disease

El síndrome de Sjögren primario (SSP) es una exocrinopatía autoinmune crónica caracterizada por infiltración linfocítica de tejido glandular y extraglandular; el compromiso pulmonar es heterogéneo; el objetivo del presente estudio es informar el primer caso en Colombia de SSP con neumonía intersticial linfocítica y enfermedad quística pulmonar, plantear diagnósticos diferenciales, abordaje diagnóstico y alternativas terapéuticas.

The primary Sjögren's syndrome (PSS) is a chronic autoimmune exocrinopathy characterized by lymphocytic infiltration of glandular and extraglandular tissue, the lung involvement is heterogeneous; the aim of this study is report the first case in Colombia of PSS with lymphocytic intersticial pneumonia and cystic pulmonary disease discuss differential diagnosis and the initial diagnostic and therapeutic work up.