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G Ital Cardiol (Rome) 2018 Nov;19(11):655-657

Cardiologia, Ospedale S. Maria della Misericordia, Perugia.

Ischemic heart disease can be caused by multiple factors. However, epidemiological studies have evidenced an association between hypereosinophilia and acute coronary syndrome, most frequently observed in the Kounis and Churg-Strauss syndromes. We here report the case of a 37-year-old man, who was admitted to our hospital for acute coronary syndrome, complicated by hypokinetic cardiac arrest with severe hypereosinophilia. Read More

Semin Respir Crit Care Med 2018 Aug 7;39(4):471-481. Epub 2018 Nov 7.

Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Université Paris Descartes, Paris, France.

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is a systemic necrotizing vasculitis of small- and medium-size vessels, characterized by asthma and blood eosinophilia. EGPA typically occurs in patients with preexisting asthma, and involves the skin, lungs, and peripheral nerves. Poor-prognosis factors (i. Read More

Semin Respir Crit Care Med 2018 Aug 7;39(4):425-433. Epub 2018 Nov 7.

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.

Vasculitides are a heterogeneous group of disorders in which inflammation of blood vessel walls is present at least some time during the course of the disease. Vasculitides can affect any caliber or type of vessel in many anatomic sites; however, the disease can alter more than just vasculature. Given the diversity of vasculitides, in 2012, a revised classification system was proposed to categorize vasculitides by the type of vessel involved including size, function, and structural attributes. Read More

J Ultrasound 2018 Oct 27. Epub 2018 Oct 27.

Department of Radiology-Ospedale del Mare-ASL NA1 Centro-Napoli, Naples, Italy.

Purpose: Typhlitis, also known as neutropenic colitis, is a rare inflammatory condition and a potentially life-threatening disease process that typically involves the cecum. Delay in diagnosis may lead to a fatal prognosis with a death rate of 21-48%. Ultrasound evaluation of right lower quadrant may lead to an accurate and rapid diagnosis. Read More

Z Rheumatol 2018 Dec;77(10):907-922

Abt. für Rheumatologie, Immunologie und Rehabilitation, Kantonsspital St. Gallen, St. Gallen, Schweiz.

Eosinophilic granulocytes form peripheral effector cells controlled by Th2 lymphocytes, which cause local cell, tissue, and functional disorders of infiltrated organs via the release of cytotoxic basic proteins and oxygen radicals. Diseases associated with eosinophilia include systemic and organ-related forms. The lungs are involved in eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome), acute and chronic eosinophilic pneumonia, as well as in an organ manifestation in hypereosinophilic syndrome and certain parasitic diseases. Read More

BMJ Case Rep 2018 Oct 25;2018. Epub 2018 Oct 25.

Westmead Clinical School, University of Sydney, Sydney, New South Wales, Australia.

Patients with Churg-Strauss syndrome often suffer from unusual cardiac manifestations and sudden cardiac death. This differs from patients with other autoimmune disorders, who typically present with premature ischaemic heart disease. We report the case of a 56-year-old man with recurrent coronary vasospasm, including an inferoposterior ST-elevation myocardial infarction, complicated by bradycardic arrest. Read More

PLoS One 2018 11;13(10):e0205768. Epub 2018 Oct 11.

Division of Rheumatology and the Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.

Objective: Identification of a biomarker for disease activity in eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss) remains an unmet need. This study examined the value of serum periostin, a marker of type 2 inflammation, as a measure of disease activity in patients with EGPA.

Methods: Participants enrolled in a multicenter, prospective cohort of patients with EGPA were included in this study if they had disease activity (defined as Birmingham Vasculitis Activity Score [BVAS] > 0) during follow-up. Read More

Joint Bone Spine 2018 Sep 7. Epub 2018 Sep 7.

Centre de référence des maladies systémiques auto-immunes rares, département de médecine interne, hôpital Cochin, Assistance publique-Hôpitaux de Paris, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France; Université Paris Descartes, 12, rue de l'École-de-Médecine, 75006 Paris, France; Institut Cochin, Inserm U1016, CNRS UMR 8104, Paris, France. Electronic address:

Targeted immunotherapy is substantially improving the management of ANCA-associated vasculitides (AAV), which include granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). This article reviews the current role for targeted immunotherapy in AAV, its validated indications, and avenues for further development. Rituximab is a validated induction treatment for GPA and severe MPA. Read More

J Allergy Clin Immunol Pract 2018 Sep - Oct;6(5):1496-1504

UNC Kidney Center, University of North Carolina at Chapel Hill, Chapel Hill, NC.

Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome) is a small vessel vasculitis associated with asthma and eosinophilia. Despite its rarity, continuous gains are being made in understanding the disease with knowledge advancements regarding its epidemiology, heterogeneous clinical manifestations, management, and outcomes. Large knowledge gaps remain, however, particularly surrounding pathophysiologic and diagnostic uncertainties. Read More

Curr Hypertens Rev 2018 Sep 3. Epub 2018 Sep 3.

Division of Cardiovascular Medicine, University of Southern California, Los Angeles-90033 CA. United States.

Acute eosinophilic myocarditis (EM) is a rare form of heart failure that is characterized by myocardial eosinophilic infiltration usually in association with peripheral eosinophilia. The underlying cause is variable and can include allergic reactions, parasitic infection, idiopathic hypereosinophilic syndrome, malignancy, Loeffler's syndrome, Churg-Strauss syndrome (CSS), early giant cell myocarditis and malignancy. The course is potentially fatal, and early diagnosis and treatment with steroids is essential. Read More

Respir Med 2018 09 24;142:66-72. Epub 2018 Jul 24.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. Electronic address:

Background: Although asthma, rhinitis/rhinosinusitis and peripheral eosinophilia are present in virtually all patients with eosinophilic granulomatosis with polyangiitis (EGPA), the role of atopy in these patients is not well defined.

Objective: To clarify the role of atopy in patients affected with EGPA.

Methods: Clinical, laboratory and standard spirometry data have been abstracted from medical records. Read More

Internist (Berl) 2018 09;59(9):898-910

Abt. für Rheumatologie, Immunologie und Rehabilitation, Kantonsspital St. Gallen, St. Gallen, Schweiz.

Eosinophilic granulocytes form peripheral effector cells controlled by Th2 lymphocytes, which cause local cell, tissue, and functional disorders of infiltrated organs via the release of cytotoxic basic proteins and oxygen radicals. Diseases associated with eosinophilia include systemic and organ-related forms. The lungs are involved in eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome), acute and chronic eosinophilic pneumonia, as well as in an organ manifestation in hypereosinophilic syndrome and certain parasitic diseases. Read More

Cureus 2018 Jun 12;10(6):e2792. Epub 2018 Jun 12.

Department of Cardiology, University of Kansa, Kansas City, USA.

Eosinophilic granulomatosis with polyangiitis (EGPA), historically known as the Churg-Strauss disease, is a small- to medium-sized vessel multi-organ vasculitis with a propensity to involve the heart. EGPA is a rare condition with an estimated annual incidence of one to 4.2 people per million. Read More

Postgrad Med 2018 Sep 2;130(7):581-596. Epub 2018 Aug 2.

a Department of Rheumatology and Internal Medicine , Poznan University of Medical Sciences , Poznan , Poland.

Background: Granulomatosis with polyangiitis (GPA) presents a wide spectrum of manifestations from the common respiratory symptoms to infrequent neurological and cardiac complications. The challenge in diagnosis and management makes the rapidly progressive disorder one of the most challenging dilemmas in clinical medicine.

Method: An extensive electronic literature search was done using multiple databases (e. Read More

Case Rep Dermatol 2018 May-Aug;10(2):175-181. Epub 2018 Jun 29.

Sector of Dermatology and Post-Graduation Course - University Hospital and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.

The authors present a classic case of Churg-Strauss syndrome with an exuberant clinical picture in a 34-year-old woman. She showed the following diagnostic criteria: asthma, polyneuropathy, rhinopathy, marked eosinophilia, positive p-ANCA with a perinuclear pattern, and skin histopathology results suggestive of vasculitis with eosinophils. There was a good response to prednisone, dexamethasone pulse therapy, and cyclophosphamide. Read More

Pediatr Pulmonol 2018 Dec 26;53(12):1640-1650. Epub 2018 Jun 26.

Pediatric Pulmonology and Allergology Department, Hôpitaux pédiatriques de Nice CHU-Lenval, Nice, France.

Objectives: To describe the characteristics of pediatric cases of eosinophilic granulomatosis with polyangiitis (EGPA), a systemic necrotizing vasculitis rarely diagnosed in children, retrieved from the French Reference Center for rare pediatric lung diseases and compared with adult cases included in the French Vasculitis Study Group cohort.

Methods: We collected information on pediatric EGPA disease presentation, management, and outcome. Cases met the Lanham criteria and/or American College of Rheumatology classification criteria. Read More

Clin Exp Allergy 2018 Oct 10;48(10):1305-1316. Epub 2018 Jul 10.

Clinical Research Center, National Hospital Organization Sagamihara National Hospital, Sagamihara, Japan.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a disease characterized by allergic granulomatosis, necrotizing vasculitis, and peripheral blood eosinophilia. Interleukin (IL)-33, thymic stromal lymphopoietin (TSLP), and type 2 innate lymphoid cells (ILC2) are involved in the innate and type 2 immune responses in EGPA. However, the relationships among these molecules and the mechanisms underlying the development of EGPA remain unknown. Read More

Iran J Med Sci 2018 May;43(3):332-335

Resident in Internal Medicine, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.

Eosinophilic granulomatosis with polyangiitis formerly named "Churg-Strauss syndrome (CSS)" is a systemic disease with bronchial asthma, hypereosinophilia, and systemic vasculitis. We report a case of CSS with cholecystitis and mononeuritis multiplex. A 50-year-old woman with a history of sinusitis and bronchial asthma of 8 years' duration was referred with a complaint of left-hand deformity and difficulty in walking. Read More

Clin Exp Rheumatol 2018 Mar-Apr;36 Suppl 111(2):12-32. Epub 2018 May 18.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.

Systemic vasculitis are heterogeneous, complex and disabling disorders. Following the previous annual reviews of this series, this paper gives a brief overview on current knowledge about recent literature on small- and large-vessel systemic vasculitis, with a specific focus on pathogenetic and clinical aspects, novel possible disease-related biomarkers and current and future therapies that are in the pipeline. Read More

Reumatol Clin 2018 May 16. Epub 2018 May 16.

Faculty of Behavioral, Management and Social sciences, Department Psychology, Health and Technology, University of Twente, Enschede, The Netherlands.

Antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss). In this report we used mycophenolate mofetil (MMF) and steroids to induce and maintain remission in two newly diagnosed cases with c-ANCA associated GPA. The two patients' maintained remission with no disease relapses during one year follow-up. Read More

Drugs 2018 May 15. Epub 2018 May 15.

Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Université Paris Descartes, Paris, France.

Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) is a rare type of anti-neutrophil cytoplasm antibody-associated vasculitis. Nevertheless, eosinophilic granulomatosis with polyangiitis stands apart because it has features of vasculitis and eosinophilic disorders that require targeted therapies somewhat different from those used for other anti-neutrophil cytoplasm antibody-associated vasculitides. Considerable advances have been made in understanding the underlying pathophysiology of eosinophilic granulomatosis with polyangiitis that have highlighted the key role of eosinophils and opened new therapeutic opportunities. Read More

Z Rheumatol 2018 Dec;77(10):928-937

Klinik für HNO-Heilkunde, Kopf- und Halschirurgie, Christian-Albrechts-Universität zu Kiel, Arnold-Heller-Str. 3, Haus 27, 24105, Kiel, Deutschland.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare granulomatous vasculitis of the small and medium sized blood vessels of unknown etiology. A regular involvement of the head and neck area is known. Since it is not yet known whether the inflammatory processes in the head and neck area are parallel to the systemic disease activity, the course of systemic and local disease activity was investigated in a comparative study. Read More

Clin Rheumatol 2018 Jun 21;37(6):1729-1735. Epub 2018 Mar 21.

Department of Vascular Problems in Rheumatic Diseases, V.A. Nasonova Research Institute of Rheumatology, Kashirskoe shosse 34A, Moscow, Russian Federation, 115522.

Recent advances in pharmacology have greatly expanded the drug repertoire for treatment of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic disorder, a type of the ANCA-associated vasculitis. Important features of this disease are eosinophilia and anti-myeloperoxidase ANCA presence in around 30-70% of patients. Read More

Clin Exp Rheumatol 2018 Mar-Apr;36 Suppl 111(2):65-72. Epub 2018 Feb 14.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.

Objectives: We estimated the cumulative patient survival rates, the causes of death and the initial predictors of death in Korean patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA).

Methods: We reviewed the medical records of 153 patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We collected clinical and laboratory data including ANCA, Birmingham vasculitis activity score (BVAS), five factor score (FFS) (2009), comorbidities, medications and prognosis (death and relapse). Read More

Int J Cardiol 2018 05 3;258:321-324. Epub 2018 Feb 3.

Service de cardiologie, Centre Hospitalier Universitaire Vaudois, Switzerland.

This report summarises three cases of Churg-Strauss syndrome (CSS) illustrating the diagnostic challenges associated with the cardiac manifestation of this disease. Here, we illustrate the role of cardiac magnetic resonance (CMR) for diagnosis and follow-up of CSS with a focus on new non-contrast T-weighted imaging sequences for quantification of myocardial scar tissue and quantitative T mapping techniques, which allow the detection of myocardial edema. Read More

Gac Med Mex 2018 ;154(1):62-67

Subdirección de Investigación Biomédica, Hospital General Dr. Manuel Gea González, Ciudad de México, México.

Cutaneous vasculities (CV) represents a diagnostic challenge, occurs as primary cutaneous disorder or as a manifestation of other entities.

Objective: To search the cause of CV. Methods: Patients with CV were prospectively evaluated. Read More

Scand J Rheumatol 2018 07 7;47(4):295-302. Epub 2018 Feb 7.

d Department of Rheumatology, Clinical Sciences Lund , Lund University , Lund , Sweden.

Objectives: To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden.

Methods: Potential cases of PSV [IgA vasculitis (IgAV, Henoch-Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu's arteritis (TAK)] were identified in a comprehensive regional healthcare register. The study area is Skåne, the southernmost county of Sweden (population 1. Read More

Turk Thorac J 2017 Jul 1;18(3):72-77. Epub 2017 Jul 1.

Department of Chest Diseases, Erciyes University School of Medicine, Kayseri, Turkey.

Objectives: Eosinophilic granulomatosis with poliangiitis (EGPA) which was previously called Churg-Strauss Syndrome, is classified into eosinophilic and vasculitic phases. To characterize the eosinophilic and vasculitic phases of the disease in terms of clinical findings, serology, and treatment.

Materials And Methods: We included 15 EGPA patients in the study. Read More

Int Arch Allergy Immunol 2018 23;175(1-2):61-69. Epub 2018 Jan 23.

Respiratory Pathophysiology Unit, Cardiothoracic and Vascular Department, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis that predominantly affects small- to medium-sized vessels. It is characterized by a wide spectrum of extrapulmonary symptoms, including sinonasal and paranasal sinus abnormalities. These are the most common features of this disease, constituting diagnostic criteria for EGPA. Read More

Am J Transplant 2018 Jun 5;18(6):1544-1547. Epub 2018 Mar 5.

Department of Cardiology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Eosinophilic granulomatosis with polyangitis (EGPA), previously referred to as Churg-Strauss syndrome, is a necrotizing small vessel vasculitis associated with eosinophilic infiltrates and extravascular granulomas. We report a case of a Caucasian woman successfully bridged to heart transplantation with a continuous flow left ventricular assist device (LVAD) who survived recurrence of EGPA in the allograft. Read More

Georgian Med News 2017 Dec(273):69-75

Donetsk National Medical University of Health Ministry of Ukraine, Lyman; State Institution "Dnipropetrovsk Medical Academy of the Ministry of Health of Ukraine", Ukraine.

The purpose of the study - investigation the separate joint lesion in systemic vasculitis, their X-ray sonographic characteristics, the correlation of the articular syndrome severity with extra-articular manifestations of the diseases, as well as aspects of the arthritis pathogenesis in this category of patients. The study included 525 patients in the ratio of the examined with Henoch-Schonlen purpura, microscopic polyangiitis, cryoglobulinemic vasculitis, polyarteritis nodosa, Takayasu's arteritis, Wegener's granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis Churg-Strauss as a 7:4:3:1:1:1:1. Joint's damage in the form of arthritis or arthralgia observed in 32-67% different groups of patients, that depending on the disease duration, the degree of the pathological process's activity, extraarticular signs severity, lung parenchyma involving and hemodynamic status in the pulmonary circulation. Read More

J Pharm Pract 2018 Apr 21;31(2):227-229. Epub 2017 Mar 21.

3 Department of Pharmacy, Levine Cancer Institute, Carolinas HealthCare System, Charlotte, NC, USA.

Ipilimumab is indicated for the treatment of melanoma in both the metastatic and adjuvant setting. Ipilimumab inhibits cytotoxic T-lymphocyte antigen 4, leading to the augmentation of T-cell activity and an antitumor immune system response. The side effect profile of ipilimumab consists of autoimmune-like events such as dermatitis, colitis, and thyroiditis. Read More

An Bras Dermatol 2017 ;92(5 Suppl 1):56-58

Dermatology Outpatient Clinic, Universidade de Mogi das Cruzes - Mogi das Cruzes (SP), Brazil.

Churg-Strauss syndrome is a rare systemic vasculitis characterized by asthma and other allergy symptoms as well as eosinophilia and necrotizing vasculitis involving small and medium-sized vessels. Its prevalence in the general population ranges from 1-3 cases per million a year, varying according to the population studied. The authors describe a case of a female patient affected by the disease with important systemic manifestations and not very florid skin lesions. Read More

Hum Vaccin Immunother 2018 04 18;14(4):958-960. Epub 2018 Jan 18.

b Division of Internal Medicine, McGill University Health Center , Montreal , Quebec , Canada.

Vaccinations have been shown repeatedly to be extremely safe with low incidence of complications. Given the rarity, these adverse events, they must be reported and examined cautiously. This case report illustrates the first case of an adult presenting with Wells syndrome that developed soon after vaccination with the thiomersal, a common preservative, containing influenza vaccine. Read More

Wiad Lek 2017;70(5):992-994

Higher State Educational Establishment Of Ukraine "Ukrainian Medical Stomatological Academy", Poltava, Ukraine.

A clinical case of Churg-Strauss syndrome has been reported on the 53-year-old female patient Ts. with bronchial asthma and allergic rhinitis. The main clinical signs and syndromes depending on the stage of the disease are presented, as well as therapeutic treatment of patients with this disease. Read More

Rev Bras Reumatol Engl Ed 2017 Nov - Dec;57(6):605-609. Epub 2016 Aug 25.

Adnan Menderes University Medical Faculty, Department of Rheumatology, Aydin, Turkey.

The first International Chapel Hill Consensus Conference was held in 1994. There have been suggestions about the nomenclature of systemic vasculitis. Important categories were added to the classification of vasculitis, and many changes were made for disease names in the second Chapel Hill Consensus Conference 2012, which were not included in the Chapel Hill Consensus Conference 1994. Read More

Am J Med Sci 2017 11 6;354(5):486-492. Epub 2017 Apr 6.

Division of Hematology, University of British Columbia, Vancouver, British Columbia. Electronic address:

Persistent eosinophilia can cause cardiac tissue damage, typically in the form of eosinophilic myocarditis, whether the underlying cause is reactive, a clonal myeloid disorder, or idiopathic hypereosinophilic syndrome (HES). Eosinophilic myocarditis ranges from mild localized disease to multifocal widespread infiltrates associated with myocardial necrosis, thrombotic complications and endomyocardial fibrosis. Systemic treatment varies widely depending on the underlying cause, so thorough investigation and precise diagnosis are essential. Read More

Rheumatol Int 2018 Apr 10;38(4):689-696. Epub 2017 Nov 10.

Department of Internal Medicine (IV), Osaka Medical College, 2-7 Daigaku-machi, Takatsuki, Osaka, 569-8686, Japan.

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by necrotizing vasculitis of small-sized vessels with extravascular granulomas and eosinophilic infiltration. The case of a 48-year-old Japanese woman with EGPA, who presented concurrently with subarachnoid hemorrhage (SAH) and coronary vasculitis, is reported. She initially presented with bronchial asthma, and then 8 months later she developed various symptoms caused by systemic eosinophilic vasculitis and was admitted to our hospital. Read More

Rheum Dis Clin North Am 2017 11;43(4):573-578

Division of Rheumatology, University of California, Zuckerberg San Francisco General Hospital, San Francisco, 1001 Potrero Avenue, Box 0868, San Francisco, CA 94143, USA. Electronic address:

Central nervous system (CNS) disease is an uncommon but significant complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and affects 3 primary areas of the CNS: the pituitary, the pachymeninges, and the CNS vasculature. Pituitary disease in uncommon, but hormonal deficiencies can be long lasting even in the face of excellent disease response. Chronic hypertrophic pachymeninigitis occurs in anti-proteinase 3-positive patients with systemic GPA and in anti-myeloperoxidase-positive patients with a milder and more limited form of the disease. Read More

Forensic Sci Med Pathol 2017 Dec 11;13(4):454-458. Epub 2017 Oct 11.

Institute of Legal Medicine, University Hospital Frankfurt, Kennedyallee 104, 60596, Frankfurt am Main, Germany.

Although myocarditis is caused by viral infections in about 50% of cases in European countries, various other causative agents are known. We report the case of a 51-year-old man who died several months after being diagnosed with asthma by his general practitioner. This diagnosis had been confirmed by a pulmonologist approximately 6 weeks before the man's death. Read More

BMJ Case Rep 2017 Sep 23;2017. Epub 2017 Sep 23.

Department of Rheumatology, Kawasaki Medical School, Kurashiki, Japan.

Corticosteroids are the first-line treatment for patients with inflammatory myopathies. Myositis can be a clinical feature of scleroderma (polymyositis-scleroderma overlap syndrome), and treatment of this syndrome is a challenge for clinicians because moderate to high doses of corticosteroids are considered a risk factor for development of acute kidney injury in affected patients. We report here the case of a 56-year-old woman with scleroderma who developed polymyositis and was successfully treated with rituximab. Read More

J Med Case Rep 2017 Sep 24;11(1):271. Epub 2017 Sep 24.

Department of Medicine, University of Utah School of Medicine, Salt Lake City, UT, USA.

Background: Eosinophilic granulomatosis with polyangiitis is a rare, necrotizing systemic vasculitis associated with asthma and hypereosinophilia. Its cause and pathophysiology are still being elucidated.

Case Presentation: We report a case of eosinophilic granulomatosis with polyangiitis in a 50-year-old Caucasian woman who presented with chest pain, dyspnea at rest, fever, and periorbital swelling. Read More

Intern Med 2017 Nov 15;56(22):3003-3008. Epub 2017 Sep 15.

Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Japan.

Objective To investigate the clinical symptoms, the physical and neurological findings, and the clinical course of neurological complications in eosinophilic granulomatosis with polyangiitis (EGPA). Methods A retrospective chart review of EGPA cases managed by two referral hospitals was performed, with a focus on the neurological findings. The study analyzed the symptoms at the onset of EGPA and investigated their chronological relationship. Read More

J Allergy Clin Immunol Pract 2017 Nov - Dec;5(6):1556-1563. Epub 2017 Sep 12.

Department of Rheumatology and Clinical Immunology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany. Electronic address:

Background: Rituximab (RTX) is approved for induction therapy of granulomatosis with polyangiitis and microscopic polyangiitis. In eosinophilic granulomatosis with polyangiitis (EGPA), organ-threatening manifestations are mainly treated with cyclophosphamide (CYC). RTX as treatment in EGPA has been described in small case series; however long-term data and the efficacy of RTX in EGPA refractory to CYC have not been reported yet. Read More

Clin J Am Soc Nephrol 2017 Oct 25;12(10):1680-1691. Epub 2017 Aug 25.

Department of Pathology and Laboratory Medicine, Department of Medicine, and Kidney Center, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.

ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). Read More

J Ophthalmol 2017 13;2017:7298026. Epub 2017 Jul 13.

Department of Ophthalmology, China-Japan Union Hospital of Jilin University, Changchun City, China.

Peripheral ulcerative keratitis (PUK) is type of crescent-shaped inflammatory damage that occurs in the limbal region of the cornea. PUK is always combined with an epithelial defect and the destruction of the peripheral corneal stroma. PUK may have a connection to systemic conditions, such as long-standing rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Wegener granulomatosis (WG), relapsing polychondritis, classic polyarteritis nodosa and its variants, microscopic polyangiitis, and Churg-Strauss syndrome. Read More

Clin Rheumatol 2017 Sep 31;36(9):2159-2162. Epub 2017 Jul 31.

GIRAT: Grupo de Investigación en Reumatología, Autoinmunidad y Medicina Traslacional, Fundación Valle del Lili and Univesidad Icesi, Cali, Colombia.

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab. Read More

Autoimmun Rev 2017 Sep 12;16(9):963-969. Epub 2017 Jul 12.

Department of Internal Medicine, Hôpital Cochin, F-75014 Paris, France; National Referral Center for Systemic and Autoimmune Diseases, Hôpital Cochin, F-75014 Paris, France; Université Paris Descartes, Faculté de Médecine Paris Descartes, F-75014 Paris, France; Université Paris Descartes, Paris 5, Paris, France; Inserm, U1016, Institut Cochin, F-75014 Paris, France. Electronic address:

Background: Although peripheral nervous system involvement is common in eosinophilic granulomatosis with polyangiitis (EGPA), central nervous system (CNS) manifestations are poorly described. This study aimed to describe CNS involvement in EGPA.

Patients And Methods: This retrospective, observational, multicenter study included patients with EGPA and CNS involvement affecting cranial nerves, brain and/or spinal cord. Read More

Exp Neurobiol 2017 Jun 14;26(3):168-171. Epub 2017 Jun 14.

Department of Neurology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul 06973, Korea.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). Read More