scholarly journals Cutaneous manifestations of ANCA-associated vasculitis and immunosuppressive therapy: cause-effect relationships (a case report)

2020 ◽  
Vol 12 (3) ◽  
pp. 79-84
Author(s):  
Madina A. Kitova ◽  
Maksim V. Maksimov ◽  
Valeriy N. Marchenko ◽  
Elena A. Bruchkus ◽  
Denis A. Davydov
Keyword(s):  
Immunosuppressive Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Secondary Infection ◽  
Skin Lesions ◽  
Cutaneous Manifestations ◽  
Multiple Organs ◽  
Anca Associated Vasculitis ◽  
Clinical Triad ◽  
The One

Granulomatosis with polyangiitis, formerly known as Wegeners granulomatosis, is an autoimmune ANCA-associated systemic vasculitis characterized by extensive damage to multiple organs and systems. Besides a typical clinical triad of ENT, lungs, and kidneys injury, various types of skin lesions can be found in 1050% of cases. A severe course of the disease and low survival of patients often requires using aggressive treatment in a form of combined immunosuppressive therapy. On the one hand, it generally improves the prognosis, and on the other is itself associated with numerous complications. One of them is a secondary infection. Skin is the second most common localization of infection after the respiratory system. Preceding skin lesions caused by vasculitis may increase the risk of infection. Thus, patients with ANCA-associated vasculitis should be carefully observed for cutaneous manifestation, both before and during the immunosuppressive therapy.

scholarly journals ANCA-associated vasculitis in a 14 years-old patient: a clinical case

2020 ◽  
Vol 27 (5) ◽  
pp. 184-194
Author(s):  
A. V. Burlutskaya ◽  
N. V. Savelyeva ◽  
N. S. Тaran
Keyword(s):  
Respiratory Failure ◽  
Immunosuppressive Therapy ◽  
Clinical Case ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Pulse Therapy ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Nasal Bleeding ◽  
Diagnostic Research

Background. ANCA-associated systemic vasculitis is a rare childhood disease. Antineutrophil cytoplasmic autoantibodies (ANCA)-related vasculitises include microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Their rarity often leads to a late diagnosis, rapid disability and high mortality in patients due to aggressive respiratory, pulmonary lesion and renal failure.Clinical Case Description. The patient suffered from a recurrent bronchoobstructive syndrome with signs of respiratory failure, obscure origin fever and chronic rhinitis with nasal bleeding for 6 months. The patient was diagnosed with obstructive bronchitis (putative bronchial asthma debut), received antibacterial therapy and inhalation bronchodilators without stable improvement during the entire period. Skin haemorrhages and arthralgia stimulated diagnostic research to establish ANCA-associated systemic vasculitis (presence of proteinase 3-specifi c ANCAs in titre 1/80). CT lung scanning revealed frosted glass foci of reduced pulmonary pneumatisation and signs of bilateral bronchoobstruction. Immunosuppressive therapy with glucocorticosteroids (methylprednisolone pulse therapy No. 3, 1000 mg intravenously on alternate days, subsequent per os administration of 1 mg/kg/day) and cyclophosphamide (500 mg intravenously once per 28 days) was prescribed. This led to the positive dynamics with eliminated fever and skin haemorrhages, as well as essentially reduced signs of respiratory failure.Conclusion. Diagnosis of systemic vasculitis is often complicated and long-term due to commonly non-specifi c debut symptoms of autoimmune disorders. In the described case, the fi rst 6 months of illness displayed intoxication and bronchoobstruction with signs of respiratory failure. Haemorrhagic rashes, arthralgias and the presence of ANCAs are proxy to vasculitis. Standard immunosuppressive therapy for ANCA-associated vasculitis improved the patient’s condition.

scholarly journals ANCA-ASSOCIATED VASCULITIS: HETEROGENEITY OF CLINICAL MANIFESTATIONS, PROGNOSIS, CURRENT OPPORTUNITIES OF PHARMACOTHERAPY

2017 ◽  
Vol 13 (1-2) ◽  
pp. 98-105
Author(s):  
O.B. Yaremenko ◽  
L.B.
Keyword(s):  
Nervous System ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Joint Involvement ◽  
Upper Respiratory Tract ◽  
Diagnosis And Prognosis ◽  
Anca Associated Vasculitis

The article highlights the new views on classification and nomenclature of systemic vasculitis, the meaning of detection of antineutrophil cytoplasmic antibodies (ANCA) in the diagnosis and prognosis of the disease. The literature data and own research regarding clinical manifestations of ANCA-associated vasculitis are presented.  Analyzing the first clinical manifestations in 41 patients with granulomatosis with polyangiitis, we identified four variants of the debut: with involvement of ENT organs (n ​=21), with lung lesions without involvement of the upper respiratory tract (n=8), with skin lesions, joint involvement and fever (n=7) and other variants (n=5). Fever (76%), involvement of ENT organs (51%), skin (41%), nervous system (39%) and arthritis/arthralgia (37%) were the most frequent first manifestations of granulomatosis with polyangiitis. In comparison with the presenting features there were more often lesions of the skin (66% vs. 41%), nervous system (51% vs. 39%), kidneys (41% vs. 10%), lungs (63% vs. 30%), eyes (32% vs. 10%) and myalgia (34% vs. 12%) throughout course of disease.  Among the lesions of the ENT organs, sinusitis (n=19), rhinitis (n=8) and otitis (n=6) were predominate, laryngotracheitis, sublottic stenosis, saddle-shaped deformation of the nose, destruction of the walls of the paranasal sinuses and mastoiditis were diagnosed less frequently. The latest clinical recommendations for treating patients with ANCA-associated vasculitis, including the use of immunobiological therapy are presented, as well as describing of the results of our own experience in the using of immunobiological therapy in patients with granulomatosis with polyangiitis.

scholarly journals Dermatologic manifestations of granulomatosis with polyangiitis: A case report and literature review

2019 ◽  
Vol 6 (4) ◽  
pp. 19
Author(s):  
Ashley Thomas ◽  
Nithya Krishnan ◽  
Nicole Vesely ◽  
Myint Thway ◽  
Rafik Jacob
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Signs ◽  
Granulomatous Inflammation ◽  
Signs And Symptoms ◽  
Organ Damage ◽  
Skin Lesions ◽  
African American Female ◽  
Cutaneous Manifestations ◽  
Serologic Testing

Granulomatosis with polyangiitis (GPA) is an ANCA-associated, systemic vasculitis of small and medium-sized blood vessels. GPA causes inflammation and destruction to the vessel wall and eventual tissue and organ damage. It classically affects the tissues and vasculature of the sinuses, lungs, and kidneys. The organ damage results in epistaxis, cough, hemoptysis, shortness of breath and/or even kidney failure. Cutaneous manifestations are rare, but have been reported. We discuss the case of a 27-year-old African American female who presented with skin lesions on her bilateral forearms and hands, several years after diagnosis and treatment for GPA. The lesions were isolated papules, located on bilateral elbows, palms, and the lateral edge of the 3rd digit on her left hand. The lesions were intensely pruritic and non-painful. A skin biopsy of the lesions showed neutrophilic and granulomatous inflammation with nuclear debris, a largely non-specific histopathologic finding. An extensive autoimmune work up revealed elevated c-ANCA/PR3-ANCA levels supporting a diagnosis of cutaneous GPA. GPA is diagnosed by a combination of clinical signs and symptoms, serologic testing, and histology from biopsy of affected organs. Patient’s who meet the criteria should be tested for anti-neutrophil cytoplasmic antibody (ANCA), specifically c-ANCA/PR3-ANCA. A positive ANCA is supportive for GPA diagnosis. However, a negative ANCA does not rule out disease. Patients with cutaneous findings suggestive of GPA and positive c-ANCA/PR3-ANCA serologic testing should be closely followed up, which will lead to overall better prognosis, improved health outcomes and reduced patient and health care expenses.

scholarly journals Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Basheer Tashtoush ◽  
Roya Memarpour ◽  
Yasmin Johnston ◽  
Jose Ramirez
Keyword(s):  
Pyoderma Gangrenosum ◽  
Granulomatosis With Polyangiitis ◽  
Leukocytoclastic Vasculitis ◽  
Systemic Vasculitis ◽  
Rare Presentation ◽  
Serological Tests ◽  
Cutaneous Manifestations ◽  
Wide Range ◽  
Common Skin ◽  
Nasal Septum Perforation

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions.

scholarly journals Subarachnoid Hemorrhage and Prostatic Involvement in Granulomatosis with Polyangiitis – An Atypical Presentation

2019 ◽  
Vol 19 (01) ◽  
pp. 34-39
Author(s):  
Raymond Bak Hei Chu ◽  
Priscilla Ching Han Wong ◽  
Joey Ka Ming Wai ◽  
Florence Hiu Yi Yap
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Vertebral Artery ◽  
Immunosuppressive Therapy ◽  
Acute Urinary Retention ◽  
Granulomatosis With Polyangiitis ◽  
Rare Complication ◽  
Artery Aneurysm ◽  
Atypical Presentation ◽  
Anca Associated Vasculitis ◽  
First Case

Granulomatosis with Polyangiitis (GPA) is a form of Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. We report a case of a 56-year-old man with GPA who first presented with acute urinary retention, and then developed pulmonarycavitating lesions and subarachnoid hemorrhage (SAH). The SAH was due to a ruptured right vertebral artery aneurysm, whichwas subsequently embolized. The patient initially had a good response to immunosuppressive therapy but later succumbed due to fulminant sepsis. SAH is a rare complication of GPA and usually not associated with a detectable aneurysm. Our patient is thesecond case in the literature where an aneurysm had been detected on angiography and the first case where examination of the culprit aneurysm revealed features of vasculitis.

scholarly journals Pediatric Granulomatosis With Polyangiitis Mimicking IgA Vasculitis: A Case Report

2020 ◽  
Vol 13 ◽  
pp. 117954412096737
Author(s):  
Vadood Javadi Parvaneh ◽  
Arezoo Shirzani ◽  
Khosro Rahmani ◽  
Reza Shiari
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
High Titer ◽  
Renal Involvement ◽  
Iga Vasculitis ◽  
Case Presentation ◽  
Tissue Biopsy ◽  
Anca Associated Vasculitis ◽  
Wide Range ◽  
Time Of Presentation

Background: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lower respiratory tract along with glomerulonephritis and is very rare in childhood. Its renal manifestations similarity with IgA vasculitis can be misleading. Case presentation: Herein, we report a 12-years-old girl with the clinical picture of IgA vasculitis and renal involvement at the time of presentation, over time, elevated cytoplasmic Anti-neutrophil Cytoplasmic Antibody (C-ANCA) and tissue biopsy confirmed GPA. Conclusion: In the case of a patient with an unusual presentation of IgA vasculitis, to some degree of suspicion, the GPA should be considered. Also, in approach to non-thrombocytopenic palpable petechia and purpura a wide range of differential diagnosis such as infections, ANCA associated vasculitis, and secondary vasculitis should be considered. Therefore, 2 effective method of GPA diagnosis, the high titer of C-ANCA test and tissue biopsy, should be considered simultaneously.

scholarly journals Kaposi sarcoma in anti-neutrophil cytoplasmic antibody-associated vasculitis: a case-based review

2021 ◽  
Author(s):  
Benedict K. Tiong ◽  
Arun S. Singh ◽  
G. Peter Sarantopoulos ◽  
Tanaz A. Kermani
Keyword(s):  
Immunosuppressive Therapy ◽  
Systemic Vasculitis ◽  
Rare Complication ◽  
Kaposi Sarcoma ◽  
Infectious Complications ◽  
Human Herpes Virus 8 ◽  
Cutaneous Manifestations ◽  
Potential Risk Factors ◽  
Clinical Stabilization ◽  
Case Based

AbstractAnti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) are systemic necrotizing vasculitides associated with significant morbidity and mortality. Given the immunosuppression used to manage these conditions, it is important for clinicians to recognize complications, especially infectious ones, which may arise during treatment. Kaposi sarcoma (KS) is a lymphoangioproliferative neoplasm caused by human herpes virus 8 (HHV-8). Its cutaneous manifestations can mimic vasculitis. We describe a 77-year-old man with microscopic polyangiitis with pulmonary-renal syndrome treated with prednisone and intravenous cyclophosphamide who developed KS (HHV-8 positive) after 2 months of treatment. Cyclophosphamide was discontinued and prednisone gradually lowered with improvement and clinical stabilization of KS lesions. This comprehensive review includes all published cases of KS in patients with AAV, with a goal to summarize potential risk factors including the clinical characteristics of vasculitis, treatment and outcomes of patients with this rare complication of immunosuppressive therapy. We also expanded our literature review to KS in other forms of systemic vasculitis. Our case-based review emphasizes the importance of considering infectious complications of immunosuppressive therapy, especially glucocorticoids, and highlights the rare association of KS in systemic vasculitis.

scholarly journals Systematic Histological Scoring Reveals More Prominent Interstitial Inflammation in Myeloperoxidase-ANCA Compared to Proteinase 3-ANCA Glomerulonephritis

2021 ◽  
Vol 10 (6) ◽  
pp. 1231
Author(s):  
Samy Hakroush ◽  
Ingmar Alexander Kluge ◽  
Philipp Ströbel ◽  
Peter Korsten ◽  
Désirée Tampe ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Clinical Presentation ◽  
Systemic Vasculitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Laboratory Findings ◽  
Treatment Regimens ◽  
Anca Associated Vasculitis ◽  
Kidney Involvement ◽  
Tubulointerstitial Lesions ◽  
Severe Deterioration

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic vasculitis, most frequently presenting as microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA). Kidney involvement is a common and severe complication of ANCA AAV which is observed in a considerable subset of patients, mainly affecting glomeruli. However, tubulointerstitial lesions have also been described in ANCA glomerulonephritis (GN). Therefore, we aim to describe active and chronic tubulointerstitial lesions in ANCA GN subtypes by systematic scoring analogous to the Banff scoring system while also utilizing clinical and laboratory findings. Methods: A total of 49 kidney biopsies with ANCA GN were retrospectively included in a single-center cohort study between 2015–2020. Results: We report that MPO-ANCA GN is associated with more severe deterioration of kidney function independent of systemic markers of AAV disease activity, and is also associated with increased proteinuria in MPO-ANCA GN and a decreased fraction of normal glomeruli. Finally, MPO-ANCA GN showed distinct, active, and chronic tubulointerstitial lesions. Conclusion: New insights into the pathophysiology of both entities, as well as differences in the clinical presentation of MPO- versus PR3-ANCA GN, could potentially pave the way for more precise treatment regimens. Therefore, it is important to understand the differences in histopathological presentation, especially in yet underestimated active tubulointerstitial lesions of ANCA GN subtypes. This research could further improve our understanding of distinct pathophysiological mechanisms.

scholarly journals POS1230 OUTCOME FOLLOWING COVID-19 INFECTION IN ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-ASSOCIATED VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 898.2-898
Author(s):  
A. Antovic ◽  
B. Lövström ◽  
A. Hugelius ◽  
O. Borjesson ◽  
A. Bruchfeld ◽  
...  
Keyword(s):  
Maintenance Therapy ◽  
Immunosuppressive Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Data Retrieval ◽  
Induction Treatment ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Upper Airways ◽  
Anca Associated Vasculitis

Background:Patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) require immunosuppressive therapy for disease control and reduction of disease relapse and may be at risk for complications during Sars-CoV-2 (COVID-19) infection.Objectives:To analyze the consequences of COVID-19 in a large cohort of AAV patients regarding occurrence, need of hospitalization, treatment at the intensive care units (ICU), or death.Methods:Data were retrieved from March 2020 to mid-January 2021 from medical records from the AAV cohort (n=233). Patients diagnosed with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) or eosinophilic granulomatosis with polyangiitis (EGPA) were included. Data included age, gender, diagnosis, ongoing immunosuppressive medication at onset of COVID-19 or at last follow-up in non-COVID individuals. Renal involvement (ever) and estimated glomerular filtration rate (eGFR) were included. COVID-19 was confirmed either by a positive PCR test in the upper airways or by serology. Severe COVID-19 was defined as need of non-invasive ventilation, ICU care, and/or death.Results:The cohort comprised of 172 patients with GPA, 50 with MPA and 11 with EGPA. There were 121 females (52%). During the study period, 20 patients (8.6%) were diagnosed with COVID-19. The median age at data retrieval in all patients was 68 years (21-93), in the COVID-19 group 63 (29-93) and 68.5 (21-90) years in the non-COVID patients.Fourty-three patients in all (18%) were hospitalized during the study period of which 11 (4.7%) due to COVID-19 infection. In all, 8 deaths occurred of which 3 were related to COVID-19.At data retrieval, 110 (47%) patients were on prednisolone treatment, 10/20 (50%) in the COVID-19 group and 100 (47%) in the non-COVID-19 group (p=0.5), with significantly higher doses in COVID-19 patients (p<0.001). In patients hospitalized with COVID-19, 6/11 (54.5%) were on prednisolone, median dose 5 mg/day (0-50). In the total group 112 (48%) were on disease modifying anti-rheumatic drugs (DMARD) and 64 (27.5%) on rituximab as maintenance therapy. Eight patients were on induction treatment with either cyclophosphamide or rituximab.Of the 20 COVID-19 cases, 8 had severe COVID-19. Of these, 2 were inactive without immunosuppressive treatment, 4 had stable disease with prednisolone (5-7.5 mg/day) in combination with DMARDs, and 2 were active treated with high dose prednisolone (25-50 mg/day) in combination with cyclophosphamide and rituximab (n=1) or rituximab (n=1).A higher proportion of patients had active AAV (p=0.03) in the severe COVID-19 then in the non-COVID group (10/213 patients).In the group with the severe COVID-19, 1/8 (12%) patient had rituximab as maintenance therapy, compared to 61/213 (28.6%) in the group of non-COVID-19 patients (p=0.5).Renal involvement (ever) was present in 144 patients (62%), in 6 patients (30%) with COVID- 19, from which 5 (62%) were in the group of severe COVID-19 patients. Median eGFR did not differ between severe COVID-19 and remaining patients with renal involvement independently of COVID-19 infection.Conclusion:We found a high rate of severe COVID-19 infection in our cohort of AAV patients which indicates risk for serious complications, especially in patients with active disease and intense immunosuppressive therapy. Maintenance therapy with rituximab did not seem to increase the risk for severe COVID-19. The findings stress the need for continued shielding and early vaccination in AAV patients.Disclosure of Interests:None declared

scholarly journals A 28-Year-Old Male Patient with Nail Tumors, Skin Lesions, and Epilepsy

2017 ◽  
Vol 9 (1) ◽  
pp. 12-19
Author(s):  
Deepak M.W. Balak ◽  
Bernard A. Zonnenberg ◽  
Juliette M.J.  Spitzer-Naaijkens ◽  
Mieke M. Hulshof
Keyword(s):  
Differential Diagnosis ◽  
Older Patients ◽  
Autosomal Dominant ◽  
Skin Lesions ◽  
Histopathological Analysis ◽  
Autosomal Dominant Disorder ◽  
Cutaneous Manifestations ◽  
Multiple Organs ◽  
Lower Back ◽  
Giant Cell Astrocytoma

Background: Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by the development of benign hamartomas in multiple organs. Most patients with TSC exhibit cutaneous manifestations. Methods: We report a 28-year-old patient with multiple pink papules at the proximal nail fold of several toes. Results: Histopathological analysis of a biopsy of a papule was consistent with an ungual fibroma. Histopathological analysis of a biopsy of an elevated skin-colored plaque at the lower back was diagnostic for a Shagreen patch. These findings were consistent with a clinical diagnosis of TSC. This patient was subsequently referred to a multidisciplinary TSC clinic for further screening, which revealed a giant cell astrocytoma and multiple subependymal tubers. Annual monitoring was recommended. The skin lesions were treated with topical rapamycin ointment. Conclusions: Recognizing dermatological manifestations of TSC is of importance to allow early diagnosis. TSC should be considered as a differential diagnosis in the case of ungual fibromas, even in older patients.

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