Antineutrophil Cytoplasmic Autoantibody-negative Pauci-immune Necrotizing Glomerulonephritis with Plasma Cell-rich Tubulointerstitial Nephritis Complicated with Pleuritis and Digital Ischemia.

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) predominantly affects small vessels. Almost all AAV patients are positive for myeloperoxidase- or proteinase 3-ANCA, and ANCA plays a crucial role in the pathogenesis of AAV. We herein report an ANCA-negative AAV patient with pauci-immune necrotizing glomerulonephritis and plasma cell-rich tubulointerstitial nephritis who was complicated with pleuritis and digital ischemia. ANCA-negative AAV is a rare clinical entity that is difficult to diagnose, and pleuritis and digital ischemia are rare manifestations of AAV. An early diagnosis and appropriate treatment are important, as any delay in the diagnosis may worsen the prognosis.

Lymphoid interstitial pneumonia in a patient with systemic lupus erythematosus: Case report and literature review.

Lymphoid interstitial pneumonia (LIP) is a rare form of interstitial pulmonary disease, which has been described in association with a wide range of autoimmune disorders. Although the association of this entity with Sjogren's syndrome is well known, only a few cases are reported in relation to systemic lupus erythematosus (SLE). The aim of this paper is to review the cases reported in literature to date, as well as to describe the characteristics of these patients including the new case presented herein. We will be focusing on the case of a 36-year-old female patient diagnosed with SLE on hydroxychloroquine treatment who develops pleuritic chest pain and progressive dyspnea after 3 years of follow-up. The chest CT scan showed pleural thickening and both multiple and bilateral micronodules. A lung biopsy was also performed, revealing an infiltration of lymphocytes, plasma cells, and histiocytes in the alveolar septa suggestive of LIP. After conducting a review of the literature, we identified seven other cases describing SLE in association with LIP. The majority of them were young women, and LIP tends to appear early in the course of the disease, even as a form of initial presentation in some cases. Symptoms included cough, dyspnea, and pleuritic pain, with the exception of one case which was asymptomatic. It is noteworthy that half of the patients were positive for anti-SSA/anti-SSB autoantibodies, and some of them also met criteria for Sjogren's syndrome. Treatment with steroids and other immunosuppressive agents improved symptoms in all of them.

Systemic lupus erythematosus in pediatric patients: Pulmonary manifestations.

The pulmonary manifestations of Systemic Lupus Erythematosus (SLE) in pediatric patients are poorly understood and the pulmonary manifestations reported from the adult population are generally extrapolated to the pediatric population. In the present work, the review of 228 files was carried out, in which the pulmonary manifestations, symptoms and antibody levels of the patients treated at the Hospital Regional de Alta Especialidad de Ixtapaluca (HRAEI), State of Mexico, Mexico, were identified. Statistical significance between groups was estimated using the Chi-square and Mann-Whitney U test. The main pulmonary manifestations identified were pleurisy (14 %), pulmonary hemorrhage (3.9 %), pulmonary thromboembolism (0.9 %), acute lupus pneumonitis (0.4 %), pulmonary arterial hypertension (0.4 %), and small lung syndrome (0.4 %). While the initial symptomatology was dyspnea with an incidence of 9.6 %, the mean oxygen saturation in the population was 96.87 %. Pleural effusion was identified as the most frequent pulmonary manifestation in radiographic changes. No statistically significant difference was found in antibody levels when comparing the groups. The most common pulmonary manifestation associated with SLE is pleurisy, however, the range of pulmonary manifestations in this type of patient can be very varied, as well as the presentation of each of them.

Chylothorax associated with primary membranous nephropathy: a case report.

BACKGROUND: Chylothorax is a state in which pleurisy is induced by chylomicron leakage due to lymphatic injury. Membranous nephropathy (MN) is one of the relatively common glomerular diseases that cause nephrotic syndrome in adults. Chylothorax at the onset of nephrotic syndrome is very rare in adult patients. CASE DESCRIPTION: We report a case of chylothorax associated with primary MN. A 64-year-old man visited the hospital complaining of lower extremity edema and dyspnea for 4 weeks. Laboratory findings showed no azotemia but hypercholesterolemia, hypoalbuminemia, nephrotic-range proteinuria, and microscopic hematuria. Chest and abdominal computed tomography (CT) revealed no ascites, venous thrombosis, or malignancy with the presence of right-side pleurisy. Biochemical analysis of the pleural fluid was consistent with chylothorax. The patient was confirmed to have MN by percutaneous kidney biopsy. An angiotensin receptor blocker, diuretics, and a hypolipidemic agent were prescribed; non-per os, total parenteral nutrition (TPN), and subcutaneous injection of octreotide were added for management of chylothorax. As serum anti-phospholipase receptor 2 antibody (Ab) concentration increased again, immunosuppressive therapy (IST) consisting of alternating monthly cycles of glucocorticoids and oral cyclophosphamide was instituted. With no improvement in chylothorax and deteriorating nutritional status despite 3 weeks of medical therapy, lymphangiography was performed, followed by thoracic duct embolization (TDE). The patient was discharged from the hospital on day 53 with clinical improvement. At 9 months after discharge, clinical remission of primary MN was achieved without recurrence of chylothorax. CONCLUSIONS: Patients with nephrotic syndrome may rarely exhibit refractory chylothorax without chylous ascites, increasing the risk of serious metabolic complications such as severe malnutrition. Therefore, upon confirming chylothorax associated with primary nephrotic syndrome, prompt radiologic intervention for lymphatic leakage must be considered in addition to specific IST.

Infection-related hospitalisation in young adults with systemic lupus erythematosus: data from the National Inpatient Sample.

INTRODUCTION: Care of young adults with SLE (YA-SLE, 18-24 years) is challenging due to major life transitions co-occurring with chronic healthcare needs. Studies have demonstrated poorer outcomes in the post-transition period. Epidemiological studies focused on serious infection-related hospitalisation (SIH) in YA-SLE are lacking. METHODS: We used National Inpatient Sample from 2010 to 2019 to study the epidemiology and outcomes of SIH for five common infections in SLE, namely sepsis, pneumonia, urinary tract infections, skin and soft tissue infections, and opportunistic infections. For time trends, we extended the dataset to cover 2000-2019. The primary outcome was the rate of SIH in YA-SLE compared with adults (25-44 years) with SLE and with young adults without SLE (YA-no SLE). RESULTS: From 2010 to 2019, we identified 1 720 883 hospital admissions with SLE in patients aged ≥18 years. Rates of SIH were similar in young adults and adults with SLE (15.0% vs 14.5%, p=0.12), but considerably higher than in the YA-no SLE group (4.2%, p<0.001). Among SLE with SIH, sepsis followed by pneumonia was the most common diagnosis. Significantly higher proportions of SIH among young adults than adults with SLE were comprised of non-white patients, belonged to the lowest income quartile and had Medicaid. However, only race/ethnicity was associated with SIH among YA-SLE. There was a higher prevalence of comorbid lupus nephritis and pleuritis among young adults compared with adults with SLE and SIH, and both comorbidities were associated with SIH in YA-SLE. Increasing rates of SIH, driven by sepsis, were seen over time. DISCUSSION: YA- SLE had similar rates of SIH to adults with SLE. While hospitalised YA-SLE differed sociodemographically from SLE adults and YA-no SLE, only race/ethnicity was associated with SIH in the YA-SLE group. Lupus nephritis and pleuritis were associated with higher SIH in YA-SLE. Among SLE with SIH, increasing trends of sepsis deserve further study.

Clinical Study of Different Treatment Methods for Tuberculous Pleuritis Complicated with Pleural Tuberculoma.

Objective: To compare the clinical efficacy and adverse drug reactions of four different schemes in the treatment of pleural tuberculoma. Methods: A total of 120 patients with pleural tuberculoma admitted to the Tuberculosis Department of our hospital from January 2018 to January 2021 were selected as the research subjects. According to different treatment methods, the patients were divided into four groups, with 30 cases in each group. They were as follows: group A received classical HRZE regimen, group B received HRZE+pleural injection, group C received HZE+rifabutin, and group D received HZE+rifabutin+pleural injection. All patients were treated intensively for 3 months and then consolidated treatment for 6 months according to the patient's condition. The absorption of lesions in the four groups at different time was compared, and the occurrences of adverse drug reactions and treatment outcomes during treatment were recorded. Results: After 3 months of treatment, compared with groups A, B, and C, the number of significantly absorbed cases and effective cases in group D increased, while the number of invalid cases decreased. However, there was no statistical significance in the absorption of lesions between the four groups (χ 2 = 8.272, P = 0.507). In addition, pairwise comparison showed no significant difference in the absorption of lesions (P > 0.05). After 9 months of treatment, there was no significant difference in the absorption of lesions among the four groups (χ 2 = 8.795, P = 0.185), but the absorption of lesions in group D was significantly better than that in group A (P < 0.05). During treatment, the incidence of adverse reactions in the four groups was significantly different (χ 2 = 8.779, P = 0.032). Pairwise comparison showed that the incidence of adverse reactions in groups C and D was significantly lower than that in group A (P < 0.05). The total treatment course of group A was 9-16 months, and 10 cases (33.33%) still had residual lesions or pleural thickening at the end of treatment. The total course of treatment in group B was 9-12 months, and 7 cases (23.33%) still had residual lesions or pleural thickening at the end of the course of treatment. The total treatment course of group C was 9-16 months, and 8 cases (26.67%) still had residual lesions or pleural thickening at the end of treatment. The total course of treatment in group D was 9-12months, and there were still 2 cases of residual lesions (6.67%) at the end of the course. Conclusions: HZE+rifabutin+pleural injection against tuberculosis therapy has a significant clinical efficacy in the treatment of pleural tuberculoma, which can more effectively improve the clinical symptoms of patients, improve the efficacy, and reduce complications, with a good prognosis, worthy of clinical promotion.

Cryptogenic Fibrosing Pleuritis with Rapidly Progressive Restrictive Ventilatory Dysfunction.

Cryptogenic bilateral fibrosing pleuritis is a rare condition, and its pathogenesis and clinical course are poorly understood, with no established therapy available. A 61-year-old man presented with bilateral pleural thickening and lymphocytic exudative effusions. The patient was diagnosed with fibrosing pleuritis with no evidence of a known etiology on a surgical pleural biopsy. Within 16 months from the onset of respiratory symptoms, restrictive ventilatory impairment progressed rapidly, resulting in hypercapnic respiratory failure requiring home oxygen and non-invasive positive pressure ventilation therapies.

Immunoglobulin G4-related Pleuritis Complicated with Minimal Change Disease.

A 70-year-old woman with bilateral pleural effusion and respiratory failure was admitted to our hospital. Nephrotic syndrome due to minimal change disease had been diagnosed four months before admission. Because blood tests and a pleural fluid analysis did not reveal the etiology of her condition, we performed a video-assisted thoracoscopic pleural biopsy. No specific thoracoscopic findings were noted. The pathological findings revealed an increase in immunoglobulin G4 (IgG4)-positive cells; IgG4-related pleuritis was diagnosed. Her pleuritis improved with oral corticosteroid therapy. A further investigation was performed on previous kidney samples; however, the etiology of the nephrotic syndrome was not IgG4-related disease but minimal change disease.

Eosinophilic Pleural Effusion in a Young Woman With Pleural Nodularity and Lytic Skeletal Lesions.

CASE PRESENTATION: A 21-year-old woman, a housewife with no known comorbidities, presented to the outpatient department with complaints of dry cough, left-sided pleuritic chest pain, modified Medical Research Council grade II breathlessness and backache. She had started developing these symptoms 1 month earlier. There was no history of fever, hemoptysis, or significant weight loss. She had no features suggestive of connective tissue disorder. There was no history of recurrent respiratory infections in the past. She was married for 1 year and had no children. Her sleep, bowel, and bladder habits were normal. No significant family history or medication history was noted.

Shrinking lung syndrome in pediatric systemic lupus erythematosus.

OBJECTIVE: To describe clinical, radiological and treatment characteristics in pediatric patients with SLS. MATERIAL AND METHODS: This is a descriptive and retrospective study in patients under 16 years old with the diagnosis of SLE complicated by SLS at the General Hospital. National Medical Center La Raza. Clinical, radiological and treatment variables were analyzed. Results are shown in frequencies and percentages. RESULTS: Data from 11 patients, 9 females and 2 males were collected. Mean age at diagnosis of SLS was 12.2 years. Age at diagnosis of SLE was 11.1 years. SLEDAI 17.3. Renal desease 72%, hematological 91%, lymphopenia 63%, mucocutaneous 72%, neurological 9%, arthritis 54%, serositis 91%, fever 81%, secondary antiphospholipid syndrome, low C3 72%, low C4 81%, positive ANA 91%, positive anti-DNA 91%. Regarding clinical manifestations of SLE: cough 81%, dyspnea 91%, hipoxemia 81%, pleuritic pain 71%, average oxygen saturation 83%. Chest X-rays findings: right hemidiaphragm affection 18%, left 63%, bilateral 18%. Elevated hemidiaphragm 91%, atelectasis 18%, pleural effusion 91%, over one third of the cardiac silhouette under the diphragm 36%, bulging diaphragm 45%, 5th. anterior rib that crosses over the diaphragm 91%. M-mode ultrasound: diaphragmatic hypomotility 100%, pleural effusion 63%. Pulmonary function tests: restrictive pattern in 45% of the cases. Treatment was with supplementary oxygen 100%, intubation 18%, antibiotics 100%, steroids 100%, intravenous immunoglobulin 54%, plasmapheresis 18%, cyclophosphamide 54% and rituximab 18%. The clinical course was favorable in 81%. CONCLUSIONS: SLS should be suspected in patients with SLE and active disease who present hipoxemia, pleuritic pain, cough, dyspnea, pleural effusion and signs of restriction on chest X-rays. Therefore, a diaphragmatic M-mode ultrasound should be performed in order to establish the diagnosis.

Pleural effusion in psoriatic arthritis patients: a case series and review of the literature.

Psoriatic arthritis (PsA) is a chronic inflammatory disease associated with psoriasis. Pulmonary involvement is a rare extra-articular manifestation of the disease characterized by rigidity of the chest wall and apical pulmonary fibrosis. Pleural effusion is uncommon in PsA. We present four cases of patients with PsA who developed pleural effusions. We report for the first time a PsA patient who was drug-naïve and developed unilateral pleuritis. We also describe one PsA case with pleuritis while he was on methotrexate (MTX) and two PsA cases on tumor necrosis factor (TNF) inhibitors. The literature review revealed six cases with pleural effusion, which were drug-induced. These patients presented pleural effusions while they were treated with MTX (2 patients) and TNF inhibitors (4 patients). In PsA patients with pleuritis, a detailed investigation to rule out infections is necessary. In addition, increased pharmacovigilance will detect cases of drug-induced serositis.

Etiological Distribution and Morphological Patterns of Granulomatous Pleurisy in a Tuberculosis-prevalent Country.

The cause of epithelioid granulomatous inflammation varies widely depending on the affected organ, geographic region, and whether the granulomas morphologically contain necrosis. Compared with other organs, the etiological distribution and morphological patterns of pleural epithelioid granulomas have rarely been investigated. We evaluated the final etiologies and morphological patterns of pleural epithelioid granulomatous inflammation in a tuberculosis (TB)-prevalent country. Of 83 patients with pleural granulomas, 50 (60.2%) had confirmed TB pleurisy (TB-P) and 29 (34.9%) had probable TB-P. Four patients (4.8%) with non-TB-P were diagnosed. With the exception of microbiological results, there was no significant difference in clinical characteristics and granuloma patterns between the confirmed TB-P and non-TB-P groups, or between patients with confirmed and probable TB-Ps. These findings suggest that most pleural granulomatous inflammation (95.2%) was attributable to TB-P in TB-endemic areas and that the granuloma patterns contributed little to the prediction of final diagnosis compared with other organs.

Levels of YKL-40 in pleural effusion and blood from patients with pleuritis.

INTRODUCTION: In recent years, there have been a significant increase in the tests and biomarkers available for pleural fluid analysis. YKL-40 is one of the inflammatory biomarkers that is used for this purpose. The aim of our study is to assess the levels and diagnostic values of YKL-40 in patients with different types of pleural effusions (PE). MATERIALS AND METHODS: This was a prospective, observational and crosssectional study. Pleural and serum YKL-40 levels were measured using enzyme-linked immunosorbent assay in 119 patients with PEs, including 23 transudates PE, 47 malignant PE, 26 parapneumonic PE (PPPE), 17 paramalignant PE (PME) and 6 tuberculous PE (TBPE). RESULT: Median pleural YKL-40 level was higher in exudates (390.3 ng/mL) than in transudates (369.5 ng/mL) (p<0.02). For a cut-off level of 378 ng/mL, it was found to predict exudates with 70% sensitivity and 64% specificity. [area under the curve (AUC)= 0.660, p= 0.01]. Median pleural YKL-40 level was highest in PMEs (407.1 ng/mL) and the lowest in transudates (369.5 ng/ mL) and high levels, with a cut-off value of 396 ng/mL, differentiated PMEs from other subgroups with 65% sensitivity and 68% specificity. (AUC= 0.680, p= 0.02). Median serum YKL-40 level was the highest in PPPEs (351.4 ng/mL) and the lowest in TBPEs (114.2 ng/mL) (p= 0.01). For a cut-off level of 284 ng/mL, it differentiated PPPEs from TBPEs with 61% sensitivity and 100% specificity (AUC= 0.830, p= 0.01). In TBPEs, pleural/serum YKL-40 ratio was strongly related with pleural ADA (r= 1, p= 0.04). CONCLUSIONS: Pleural YKL-40 may be useful for differentiating exudates and detecting PMEs. Serum YKL-40 may be good diagnostic biomarker for differentiating PPPEs and TBPEs. Additionally, measuring serum and pleural YKL-40 and pleural ADA may be reliable way to diagnose TBPEs.

54-Year-Old Man With Cough, Chest Pain, and Dyspnea on Exertion.

CASE PRESENTATION: A 54-year-old man presented with 6 months' history of dry cough and dyspnea on exertion. He also reported intermittent joint pain and orthopnea. He denied fevers, chills, and rashes. His medical history was significant for rheumatoid arthritis, for which he was taking 20 mg of prednisone daily. He had not been receiving adalimumab or methotrexate for several months. He never smoked and drank alcohol occasionally. Family history was significant for rheumatoid arthritis.

Additive effect of dabigatran and high-dose aspirin in the development of haemorrhagic pleural effusion in a patient with tuberculous pleuritis.

Tuberculous pleuritis can rarely cause haemorrhagic pleural effusion. Dabigatran etexilate can have an additive effect on increasing the risk of haemorrhage. Aspirin cannot cause major haemorrhage, but in the elderly it can cause gastrointestinal bleeding via ulceration of the gastrointestinal mucosa. We report here the case of a 77-year-old male who presented to the hospital with a 2-month history of progressive dyspnoea. He had been taking dabigatran etexilate (220 mg) and high-dose acetylsalicylic acid (aspirin; 300 mg) daily for chronic atrial fibrillation. A chest X-ray revealed a moderately sized right pleural effusion confirmed by a computed tomography scan, which also showed bronchiectasis of both lungs. Dabigatran was discontinued and aspirin was decreased to the minimal therapeutic dose of 100 mg before thoracentesis was performed. Lymphocyte-predominant (50%) haemorrhagic fluid of 500 ml was drained, positive for acid-fast bacilli smear and polymerase chain reaction of Mycobacterium tuberculosis. A chest tube was placed and an additional 1250 ml of haemorrhagic exudate drained out. We treated the patient with a routine regimen of antituberculous medication and the infection resolved without complications other than the bronchiectasis present before treatment. We think that the combination of dabigatran etexilate and high doses of aspirin increased the risk of pleural haemorrhage in this patient with tuberculous pleuritis.

Chronic pleuritis leading to severe pulmonary restriction: a rare complication of Degos disease.

This case demonstrates chronic fibrosing pleuritis, as a rare pulmonary aetiology for mortality in patients with Degos disease or malignant atrophic papulosis (MAP). Knowledge of this unusual complication will help physicians identify this entity early and provide appropriate treatment.Patients with MAP die from gastrointestinal and brain involvement within 2-3 years of diagnosis. This case is unique as the patient survived for 9 years and died secondary to respiratory failure, which had not been reported before. Our patient was a young man, diagnosed with MAP at the age of 17. His skin and gastrointestinal disease were controlled with eculizumab and parenteral treprostinil. The patient developed severe restrictive pulmonary disease, required ventilatory support, and died from respiratory failure. An autopsy revealed chronic fibrosis pleuritis. Longer surviving patients with MAP might suffer from significant respiratory disease. Pulmonary function test should be obtained to identify subclinical respiratory limitation.