- Prevalence of overlap of antineutrophil cytoplasmic antibody associated vasculitis with systemic autoimmune diseases: an unrecognized example of poliautoimmunity. [Journal Article]
- CRClin Rheumatol 2018 Jul 14
- We aimed to estimate the frequency of overlap of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with systemic autoimmune diseases. Retrospective single-center study to identif...
We aimed to estimate the frequency of overlap of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with systemic autoimmune diseases. Retrospective single-center study to identify patients with AAV diagnosis and concomitant autoimmune systemic diseases, simultaneously, before or after the diagnosis of AAV. Sociodemographic characteristics, such as comorbidities; follow-up time; type of AAV; disease duration; relapses; treatment and response; clinical, serological, and histological characteristics; disease activity and damage; prognosis; dialysis requirements, and death were assessed. Twenty-eight of two hundred and forty-seven patients (11.3%) with AAV had a concomitant diagnosis of autoimmune disease. The predominant AAV type was renal-limited vasculitis (39%), followed by granulomatosis with polyangiitis (29%), mycroscopic polyangiitis (25%), and eosinophilic granulomatosis with polyangiitis (7%). Mean age at AAV diagnosis was 50 ± 17 years and 24/28 were ANCA positive. The main clinical manifestations were renal (79%), otorhinolaryngologic (43%), and pulmonary and peripheral neuropathy (32%). Sixteen patients (57%) experienced partial or total remission at a median follow-up of 34 months, and four patients (14%) died. The most frequent autoimmune disease overlapped was rheumatoid arthritis (39%), followed by Sjögren's syndrome and systemic sclerosis (14%), mixed connective tissue disease (11%), systemic lupus erythematosus and juvenile idiopathic arthritis (7%), and ankylosing spondylitis and IgG4-related disease (4%). In nine patients (32%), both diagnoses were simultaneous; in the rest, median time elapsed between the autoimmune disease and AAV diagnosis was 173 months. The prevalence of overlap AAV with other autoimmune diseases was low. The most common AAV phenotype was renal-limited vasculitis, and the most frequent overlap disease was rheumatoid arthritis.
- Gastrointestinal Manifestations of Hypereosinophilic Syndromes and Mast Cell Disorders: a Comprehensive Review. [Review]
- CRClin Rev Allergy Immunol 2018 Jul 12
- Hypereosinophilic syndrome and mastocytosis are relatively rare proliferative diseases encountered in the general population. However, allergists frequently consider these disorders in the differenti...
Hypereosinophilic syndrome and mastocytosis are relatively rare proliferative diseases encountered in the general population. However, allergists frequently consider these disorders in the differential of patients presenting with gastrointestinal, pulmonary, cutaneous, and allergic symptoms. Gastrointestinal symptoms are some of the most frequent and/or debilitating aspects of both disease states and in many cases lead to poor quality of life and functional limitation for the patient. They are the third most common clinical manifestation in hypereosinophilic syndrome and have been found to be the most distressful aspect of the disorder in those with systemic mastocytosis. Both eosinophils and mast cells play integral parts in normal gut physiology, but when and how exactly their effector functionality translates into clinically significant disease remains unclear, and the available literature regarding their pathophysiology remains sparse. Eosinophils and mast cells even, in fact, may not necessarily function in isolation from each other but can participate in bidirectional crosstalk. Both are affected by similar mediators and can also influence one another in a paracrine fashion. Their interactions include both production of soluble mediators for specific eosinophil and mast cell receptors (for example, eosinophil recruitment and activation by mast cells releasing histamine and eotaxin) as well as direct physical contact. The mechanistic relationship between clonal forms of hypereosinophilia and systemic mastocytosis has also been explored. The nature of gastrointestinal symptomatology in the setting of both hypereosinophilic syndrome and mast cell disease is frequently manifold, heterogeneous, and the lack of better targeted therapy makes diagnosis and management challenging, especially when faced with a substantial differential. Currently, the management of these gastrointestinal symptoms relies on the treatment of the overall disease process. In hypereosinophilia patients, systemic corticosteroids are mainstay, although steroid-sparing agents such as hydroxyurea, IFN-α, methotrexate, cyclosporine, imatinib, and mepolizumab have been utilized with varying success. In mastocytosis patients, anti-mediator therapy with antihistamines and mast cell stabilization with cromolyn sodium can be considered treatments of choice, followed by other therapies yet to be thoroughly studied, including the role of the low-histamine diet, corticosteroids, and treatment of associated IBS symptoms. Given that both eosinophils and mast cells may have joint pathophysiologic roles, they have the potential to be a combined target for therapeutic intervention in disease states exhibiting eosinophil or mast cell involvement.
- Diffuse Alveolar Hemorrhage Induced by Vaping. [Journal Article]
- CRCase Rep Pulmonol 2018; 2018:9724530
- There has been a significant increase in electronic cigarette (e-cigarette) use since its introduction in 2007. Ironically, there remains very few published literature on the respiratory complication...
There has been a significant increase in electronic cigarette (e-cigarette) use since its introduction in 2007. Ironically, there remains very few published literature on the respiratory complications of e-cigarettes. The use of personalized vaporizers or commonly known as "vaping" has started to overtake standard e-cigarette. Its dynamic vaporizer customization makes it challenging to assess long-term health effects. Case reports on the pulmonary complications of e-cigarettes are limited to bronchiectasis, eosinophilic pneumonia, pleural effusion, and suspected hypersensitivity pneumonitis. Diffuse alveolar hemorrhage (DAH) is bleeding into the alveolar spaces of the lung secondary to disruption of the alveolar-capillary basement membrane. We report a case of young male presenting with subacute respiratory failure. He was later found to have diffuse alveolar hemorrhage syndrome that is likely induced by aggressive vaping. This adds up to the rising concern on the possible serious complications of this innovative technology designed as a safer alternative to traditional cigarettes.
- The Respiratory System in Autoimmune Vascular Diseases. [Journal Article]
- RRespiration 2018; 96(1):12-28
- The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophili...
The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Pulmonary and ENT involvements are the hallmark feature of the disease and are present in 90 and 80% of cases, respectively, with frequent skin or gastrointestinal involvement. In about 10% of cases, the lung is the only organ affected. Eosinophilic granulomatosis with polyangiitis is always associated with hypereosinophilia and asthma which usually precedes the systemic manifestations by several years; however, onset of asthma and of the vasculitis may be concomitant. Parenchymal infiltrates may be migratory and rapidly resolve upon corticosteroid treatment. Diffuse alveolar hemorrhage and renal failure are typical features of microscopic polyangiitis. The former is the leading manifestation of anti-glomerular basement membrane disease and is usually part of a pulmonary-renal syndrome. Takayasu arteritis has a distinct clinical presentation due to pulmonary arteritis and may present with massive hemoptysis, chest pain, and rarely symptoms of pulmonary hypertension. Behçet disease is the most common cause of pulmonary artery aneurysm and can also cause in situ thrombosis of the pulmonary arteries. Corticosteroids and immunosuppressive agents are the mainstay of treatment. In conclusion, systemic vasculitis is a frequent cause of respiratory system involvement with diverse manifestations of distinct severity and outcome.
- Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort. [Journal Article]
- PPPediatr Pulmonol 2018 Jun 26
- CONCLUSIONS: This French cohort is the biggest pediatric EGPA series described to date, with a long follow-up period. The findings confirm that pediatric EGPA has specific clinical, radiological, and histological characteristics that differ from adult EGPA. Development of systemic symptoms, and consequently diagnosis, occur with a shorter delay in children, mainly during the eosinophilic phase and leading to a specific presentation.
- Pediatric Case Report on an Interstitial Lung Disease with a Novel Mutation of SFTPC Successfully Treated with Lung Transplantation. [Journal Article]
- JKJ Korean Med Sci 2018 May 28; 33(22):e159
- Mutations of the surfactant protein (SP)-C gene (SFTPC) have been associated with neonatal respiratory distress syndrome (RDS) and childhood interstitial lung disease (ILD). If accurate diagnosis and...
Mutations of the surfactant protein (SP)-C gene (SFTPC) have been associated with neonatal respiratory distress syndrome (RDS) and childhood interstitial lung disease (ILD). If accurate diagnosis and proper management are delayed, irreversible respiratory failure demanding lung transplantation may ensue. A girl was born at term but was intubated and given exogenous surfactant due to RDS. Cough and tachypnea persisted, and symptoms rapidly progressed at 16 months of age despite treatment with antibiotics, oral prednisolone, methylprednisolone pulse therapy, and intravenous immunoglobulin. At 20 months, she visited our hospital for a second opinion. A computed tomography scan showed a diffuse mosaic pattern with ground-glass opacity and subpleural cysts compatible with ILD. A video-assisted thoracoscopic lung biopsy revealed ILD with eosinophilic proteinaceous material and macrophages in the alveolar space. Bilateral lung transplant from a 30-month-old child was done, and she was discharged in room air without acute complications. Genetic analysis revealed a novel c.203T>A, p.Val68Asp mutation of SP-C, based on the same exon as a known pathogenic mutation, p.Glu66Lys.
- Hypereosinophilic Syndrome in the Differential Diagnosis of Pulmonary Infiltrates with Eosinophilia. [Review]
- AAAnn Allergy Asthma Immunol 2018 May 24
- A Case of Pulmonary Hypertension Associated with Idiopathic Hypereosinophilic Syndrome. [Journal Article]
- IHInt Heart J 2018 May 23
- Hypereosinophilic syndrome (HES) is characterized by multi-organ damage that is associated with tissue hypereosinophilia. A persistently elevated eosinophilic count is also required for the diagnosis...
Hypereosinophilic syndrome (HES) is characterized by multi-organ damage that is associated with tissue hypereosinophilia. A persistently elevated eosinophilic count is also required for the diagnosis of HES. Although HES affects various organs, damage to pulmonary artery is rarely reported. We present a case of a 39-year-old man who was diagnosed with pulmonary hypertension (PH) associated with idiopathic HES. Although the pulmonary arterial hypertension specific drugs including intravenous epoprostenol could not control his PH, corticosteroid was effective for both hypereosinophilia and PH. Our case suggests the importance of steroid therapy as well as specific drugs for pulmonary arterial hypertension in the treatment of PH associated with HES.
- Hypersensitivity Pneumonitis and Acute Respiratory Distress Syndrome From E-Cigarette Use. [Journal Article]
- PedPediatrics 2018; 141(6)
- Electronic cigarette (e-cigarette) use, or "vaping," is gaining widespread popularity as an alternative to conventional cigarettes among adolescents. Little is known of the health risks of e-cigarett...
Electronic cigarette (e-cigarette) use, or "vaping," is gaining widespread popularity as an alternative to conventional cigarettes among adolescents. Little is known of the health risks of e-cigarette use, especially in children and adolescents. We present a Case Report of a previously healthy 18-year-old woman who presented with dyspnea, cough, and pleuritic chest pain after e-cigarette use. She developed respiratory failure with hypoxia and was intubated, and ultimately met diagnostic criteria for acute respiratory distress syndrome. Chest tubes were placed to drain worsening pleural effusions. Computed tomography of the chest revealed dependent opacities in both lung bases, superimposed smooth interlobular septal thickening, and pleural effusions. Bronchoalveolar lavage revealed cellular debris and reactive mononuclear cells, and cell counts were remarkable for elevated mononuclear cells and eosinophilia. After the results of a workup for an infectious etiology came back negative, the patient was diagnosed with hypersensitivity pneumonitis and intravenous methylprednisolone therapy was initiated. After this the patient rapidly improved, was weaned off vasopressor support, and was extubated. This is the first reported case of hypersensitivity pneumonitis and acute respiratory distress syndrome as a risk of e-cigarette use in an adolescent, and it should prompt pediatricians to discuss the potential harms of vaping with their patients. Hypersensitivity pneumonitis, lipid pneumonia, and eosinophilic pneumonia should be included in the differential diagnosis of patients who exhibit respiratory symptoms after the use of an e-cigarette.
New Search Next
- Unravelling the NERDS syndrome. [Journal Article]
- BCBMJ Case Rep 2018 May 12; 2018
- A 22-year-old man presented with symmetric polyarthritis, pruritus and deviation of angle of mouth to the right side since the last 7 years. His symptoms were persistent despite receiving ayurvedic m...
A 22-year-old man presented with symmetric polyarthritis, pruritus and deviation of angle of mouth to the right side since the last 7 years. His symptoms were persistent despite receiving ayurvedic medications and symptomatic therapy. Examination revealed dry skin, cutaneous nodules, xanthelasma, periarticular non-tender swellings, pitting oedema of hands and feet and lower motor neuron type right facial palsy. Haematological investigations revealed eosinophilia and skin biopsy had cutaneous eosinophilic infiltration. The constellation of above findings comprises the nodules, eosinophilia, rheumatism, dermatitis and swelling syndrome. It a rare syndrome with few reported cases in literature. The patient was started on oral corticosteroids which was subsequently tapered and methotrexate therapy. His polyarthritis and skin rashes resolved with therapy. He has been followed-up for 2 years and is presently asymptomatic for the last 1 year.