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Introduction: Anti-neutrophil cytoplasmic antibody (ANCA)- associated vasculitis (AAV) is a rare group of systemic autoimmune diseases that primarily target small blood vessels. Renal manifestations often present as pauci-immune focal and segmental necrotizing crescentic glomerulonephritis (PI-NCGN), which can progress to acute or chronic kidney failure and multiorgan involvement. It is frequently associated with poor outcomes. We report a case of PR3-positive ANCA-associated vasculitis complicated by rapidly progressive glomerulonephritis, acute kidney injury, and diffuse alveolar hemorrhage during the COVID-19 pandemic. This case highlights the diagnostic challenges of differentiating AAV from conditions associated with SARS-CoV-2 infection, as the clinical and radiological presentations of pulmonary-renal syndromes may overlap. The findings underscore the importance of maintaining a comprehensive differential diagnosis in patients with pulmonary and renal involvement, particularly in the post-COVID-19 era, to ensure timely and accurate management of rare autoimmune conditions such as AAV. Conclusion: ANCA-associated vasculitis (AAV) remains a diagnostic and therapeutic challenge, particularly in the post-COVID-19 era, where overlapping clinical and radiological features with SARS-CoV-2 complications can obscure timely identification. This case highlights the critical importance of maintaining a broad differential diagnosis in patients presenting with pulmonary-renal syndromes to differentiate AAV from more common conditions associated with COVID-19.

The term “Pulmonary-renal syndrome” is a clinical syndrome, which encompasses a group of disorders with distinctive clinical and radiological features suggestive of diffuse alveolar hemorrhage and glomerulonephritis. The most common etiologies implicated are antineutrophil cytoplasmic antibody ANCA ANCA-associated vasculitis and Goodpasture syndrome. Early diagnosis and prompt treatment are critical for the management as patients can land up in severe respiratory distress and renal failure. A multi-disciplinary team is required for its management. The mainstay of treatment includes steroids, immunosuppressants, and plasmaphereses, depending on the etiology. Despite optimal management, mortality and morbidity remain high.

Pulmonary-renal syndrome is called alveolar hemorrhage accompanied by glomerulonephritis. The above condition can occur as a manifestation of ANCA-associated granulomatosis with vasculitis. The course of the disease varies from mild, limited inflammation to rapid progression with multi-organ changes that can be life-threatening. The mainstay of therapy is immunosuppression - both in the acute phase of the disease and after clinical improvement. Unfortunately, common causes of death in addition to complications of the disease include side effects of immunosuppressive treatment. A 72-year-old man is transferred to the Nephrology Unit for acute kidney injury of unclear etiology. On the basis of clinical examination, laboratory and imaging studies, a diagnosis of systemic small vessel inflammation with c-ANCA occupying the kidneys, lungs and intestines was made. Due to acute respiratory failure, the patient was transferred to the ICU, where renal replacement therapy, plasmapheresis, steroid pulses and cyclophosphamide were administered. After clinical improvement, it was decided to continue cyclophosphamide therapy and monitor blood IgG levels. Unfortunately, 4 months later, the patient died of multiple organ failure caused by septic shock of bacterial etiology. In the described case, age and comorbidities were the biggest risk factors for infectious complications of immunosuppression. The population is aging, so it is necessary to analyze the current standards of immunosuppressive treatment to improve the prognosis of the elderly.

Honeybee stings with features of envenomation (either local or allergic and rarely systemic toxicity) are often seen in agriculture workers. An initial presentation with severe diffuse alveolar hemorrhage or pulmonary-renal syndrome is rare and only confined to a few case reports. Herein, we describe a case of a 45-year-old male who presented with multiple bee stings and subsequently developed acute kidney injury and pulmonary hemorrhage. He was managed with hemodialysis, invasive mechanical ventilation, red-cell transfusion, and pulse methylprednisolone. However, he developed cardiac arrhythmias with ventricular tachycardia and died. [ABSTRACT FROM AUTHOR]

Levamisole is an anti-helminthic drug with immunomodulatory properties that is added to cocaine to increase its potency and weight. Levamisole-adulterated cocaine (LAC) may cause an antineutrophil cytoplasmic antibody (ANCA)-associated systemic small vessel vasculitis (AAV). We aimed to characterize the phenotype of persons developing pulmonary-renal syndrome (PRS) in LAC-induced AAV and summarize its treatment and outcomes. Pubmed and Web of Science were searched (until September 2022). Reports that described co-existing diffuse alveolar hemorrhage and glomerulonephritis in an adult (age ≥ 18) with confirmed or suspected LAC exposure were included. Reports, demographics, clinical and serologic features, treatment and outcome characteristics were extracted. Of the 280 records identified, eight met the inclusion criteria, including eight unique cases. Persons were aged 22–58 years, and 50% were women. Cutaneous involvement occurred in only half of the cases. Other associated vasculitis findings and serologies were heterogeneous. All patients received immunosuppression with steroids, with cyclophosphamide and rituximab commonly added. We concluded that PRS could occur from LAC-induced AAV. Distinguishing LAC-induced AAV from primary AAV is challenging as clinical and serologic presentations overlap. Asking about cocaine use is requisite in persons presenting with PRS to guide diagnosis and appropriately counsel on cocaine cessation in conjunction with immunosuppression as treatment. [ABSTRACT FROM AUTHOR]

IgA nephropathy is a renal-limited form of systemic vasculitis, and pulmonary manifestations are uncommon. An initial presentation with severe diffuse alveolar hemorrhage (DAH) or pulmonary-renal syndrome is rare and only confined to a few case reports. Herein, we present a young male admitted with acute-onset dyspnea, hemoptysis, and rapidly progressive renal failure. With an initial diagnosis of an immune-mediated pulmonary-renal syndrome, he was treated with high-dose corticosteroids and therapeutic plasmapheresis along with intensive organ support (including hemodialysis, red cell transfusion, and high-flow oxygen). After a detailed laboratory evaluation and kidney biopsy, IgA nephropathy was diagnosed. The patient continued to worsen with persistent DAH and died. IgA nephropathy-associated severe DAH or pulmonary-renal syndrome is rare but increasingly recognized. The condition is difficult to diagnose early and has no proven disease-targeted therapy. [ABSTRACT FROM AUTHOR]

Anti-glomerular basement membrane (anti-GBM) disease is an acute and life-threatening systemic autoimmune disorder. The coexistence of circulating anti-neutrophil cytoplasmic antibodies (ANCA) and anti-GBM disease, the so-called double-positive disease (DPD), is exceptionally rare. We report a unique case of DPD manifesting as pulmonary-renal syndrome (PRS) in a 46-year-old woman who first presented with clinical and radiological suspicion of pneumonia. Chest computed tomography scan later revealed bilateral alveolar hemorrhage. Kidney biopsy showed necrotizing crescentic (100% glomeruli) glomerulonephritis. On immunofluorescence microscopy, glomeruli were global linear positive for IgG, confirming anti-GBM disease. Double positivity was detected for circulating anti-myeloperoxidase ANCA (p-ANCA) and anti-GBM antibodies. Acute renal failure evolved rapidly. Therapeutic plasma exchange (TPE) and hemodialysis (HD) were initiated early in combination with intravenous pulse corticosteroid therapy followed by oral methylprednisolone and cyclophosphamide. Pulmonary hemorrhage resolved, but renal function could not be preserved. The patient remains HD dependent. This case report highlights that pulmonary symptomatology may be the leading clinical presentation of PRS, with initially normal renal function at DPD onset. Early recognition and diagnosis are therefore crucial to timely clinical intervention. The role of prompt kidney biopsy and initiation of TPE and HD in PRS must not be underestimated.

Abstract Pulmonary‐renal syndrome (PRS) is defined as a combination of diffuse alveolar haemorrhage and glomerulonephritis. An 18‐year‐old woman visited our hospital with a 2‐day history of fever, dyspnoea, and leg edema. Laboratory investigations revealed an elevated inflammatory reaction, increased serum creatinine levels, and normocytic anaemia. Additionally, the anti‐streptolysin‐O titre was positive, and complement component‐3 levels were decreased. Urinalysis revealed proteinuria and hematuria. Bronchoalveolar lavage aliquots were progressively more hemorrhagic. These findings supported a diagnosis of PRS secondary to streptococcal infection. The patient was treated with high‐dose methylprednisolone and antibiotics. After 4 days of treatment, her respiratory symptoms and serum creatinine levels improved. Steroid tapering was performed over 15 days. The findings in this case indicate that streptococcal infection is a potential cause of PRS, and that short‐term steroid therapy is an effective treatment.

Cryoglobulinemia (CG) is defined as the presence of abnormal immunoglobulins (Igs)that precipitate at low temperatures and dissolve upon warming. The manifestations in type I cryoglobulinemia are often related to intravascular obstruction which include skin, joint, renal and neurological involvement. We report a rare case of type 1 CG that presented with diffuse alveolar hemorrhage. Following extensive workup, the patient was found to have membranoproliferative glomerulonephritis secondary to type 1 CG in the setting of marginal B cell lymphoma. He was started on an aggressive regimen targeting underlying lymphoma. Key to managing this condition was multidisciplinary approach towards the diagnosis and management of this otherwise challenging case.

Abstract Background Hydralazine is a common vasodilator which has been used for the treatment of hypertension and heart failure. Hydralazine can induce antineutrophil cytoplasmic antibody-associated vasculitis due to its auto-immunogenic capability and one of the very rare presentations is pulmonary–renal syndrome. Case presentation We report a case of a 64-year-old African American woman, who presented to our emergency room with shortness of breath, orthopnea, paroxysmal nocturnal dyspnea, leg swelling, fatigue, loss of appetite, cough with clear sputum, and lightheadedness. On admission, she developed acute hypoxic respiratory failure requiring intubation and acute renal failure requiring hemodialysis. A serologic workup was positive for antineutrophil cytoplasmic antibody, antinuclear antibody, anti-histone, anti-cardiolipin IgM, and anti-double-stranded DNA antibodies. A renal biopsy was done due to persistent deterioration in kidney function and demonstrated classic crescentic (pauci-immune) glomerulonephritis. Hydralazine was empirically discontinued early in the admission and she was started on corticosteroids and cyclophosphamide following biopsy results. She was clinically stable but remained dependent on hemodialysis after discharge. Conclusion Hydralazine-induced antineutrophil cytoplasmic antibody-associated vasculitis with pulmonary–renal syndrome is a rare occurrence. In the setting of hydralazine use, multiple positive antigens, and multisystem involvement, clinicians should consider this rare condition requiring prompt cessation of offending drug, early evaluation with biopsy, and contemplate empiric immunosuppressive therapy while biopsy confirmation is pending.

Pulmonary‐renal syndrome (PRS) is defined as a combination of diffuse alveolar haemorrhage and glomerulonephritis. An 18‐year‐old woman visited our hospital with a 2‐day history of fever, dyspnoea, and leg edema. Laboratory investigations revealed an elevated inflammatory reaction, increased serum creatinine levels, and normocytic anaemia. Additionally, the anti‐streptolysin‐O titre was positive, and complement component‐3 levels were decreased. Urinalysis revealed proteinuria and hematuria. Bronchoalveolar lavage aliquots were progressively more hemorrhagic. These findings supported a diagnosis of PRS secondary to streptococcal infection. The patient was treated with high‐dose methylprednisolone and antibiotics. After 4 days of treatment, her respiratory symptoms and serum creatinine levels improved. Steroid tapering was performed over 15 days. The findings in this case indicate that streptococcal infection is a potential cause of PRS, and that short‐term steroid therapy is an effective treatment. [ABSTRACT FROM AUTHOR]

Abstract Hydralazine‐induced ANCA‐associated vasculitis is a rare clinical entity, with complications including rapidly progressive glomerulonephritis, pulmonary hemorrhage, and pulmonary‐renal syndrome. We present this case to highlight the clinical features that support this challenging diagnosis and to emphasize the importance of prompt recognition and aggressive intervention given its significant morbidity and mortality.

Hydralazine‐induced ANCA‐associated vasculitis is a rare clinical entity, with complications including rapidly progressive glomerulonephritis, pulmonary hemorrhage, and pulmonary‐renal syndrome. We present this case to highlight the clinical features that support this challenging diagnosis and to emphasize the importance of prompt recognition and aggressive intervention given its significant morbidity and mortality. [ABSTRACT FROM AUTHOR]

In the article, we report the causes of pulmonary hemorrhage (PH) according to the literature data and own experience, with an emphasis on patients suffering from rheumatic diseases. Methods of diagnosis and modern approaches to the treatment of PH are analyzed.

The article analyzes the diagnosis and treatment of anti-GBM antibody disease (Goodpasture's syndrome) - a rare, severe progressive disease, associated with anti-glomerular basement membrane antibody-induced pulmonary hemorrhage and glomerulonephritis. The main problem of this pathology is late diagnosis, resulted in ineffective treatment. The article provides current information on the epidemiology, etiology and pathogenesis, diagnosis, and treatment of Goodpasture’s syndrome, as well as clinical case of a patient with this rare disease.

Leukocyte chemotactic factor-2 (LECT2) amyloidosis has been described as being associated with kidney disease; however, no clinical manifestations outside of the kidney have been previously reported. We describe a patient presenting with pulmonary-renal syndrome found to have deposition of amyloidogenic LECT2 (ALECT2) within both the lung and the kidney. This case is unique in regard to both the patient's clinical presentation of pulmonary-renal syndrome in the setting of amyloidosis and the biopsy finding of ALECT2 deposition within the lung. It also emphasizes the importance of tissue diagnosis in such cases, given that amyloidosis was not initially considered in the differential diagnosis.

Abstract Background Anti-glomerular basement membrane (anti-GBM) disease is characterized by circulating IgG glomerular basement membrane antibodies and is clinically expressed as a rapidly progressive crescentic glomerulonephritis (GN), with 30–60% of patients also developing pulmonary hemorrhage. Classically, the renal biopsy shows glomerular crescent formation, bright linear staining of glomerular basement membranes (GBM) for IgG on direct immunofluorescence (IF), and the serologic presence of circulating anti-GBM antibodies. Recently, patients with linear IgG IF staining, undetectable circulating anti-GBM antibodies and glomerular changes atypical for anti-GBM disease have been described as “atypical anti-GBM disease”, with a distinctly more benign clinical course than typical anti-GBM disease. We present a case report of a patient with negative anti-GBM serology but positive linear IgG staining by IF, severe diffuse crescentic and endocapillary proliferative glomerulonephritis, and renal failure, complicated by severe pulmonary hemorrhage after immunosuppression, likely due to cytomegalovirus (CMV) pneumonitis. Case presentation A 24-year-old man was admitted to hospital with hemoptysis and renal failure. Investigations for anti-GBM serology by addressable laser bead immunoassay (ALBIA) was negative for anti-GBM antibodies. Renal biopsy showed diffuse endocapillary proliferative glomerulonephritis with membranoproliferative features and diffuse circumferential crescents. Direct IF showed strong linear staining for IgG along GBMs. The patient’s hemoptysis improved with immunosuppression, but 1 month later he was readmitted with gross hemoptysis, which was refractory to further cyclophosphamide, plasma exchange and rituximab. Bronchoalveolar lavage (BAL) and blood work confirmed CMV pneumonitis, and the patient’s hemoptysis resolved with ganciclovir, though he became dialysis dependent. Conclusions This case demonstrates an atypical presentation of anti-GBM disease with both crescents and endocapillary hypercellularity and negative serology. The patient is dialysis dependent, unlike most previously described patients with atypical anti-GBM disease. The course was complicated by CMV pneumonitis, which contributed to the severity of the pulmonary manifestations and added diagnostic difficulty.

Background: Hydralazine is a common vasodilator which has been used for the treatment of hypertension and heart failure. Hydralazine can induce antineutrophil cytoplasmic antibody-associated vasculitis due to its auto-immunogenic capability and one of the very rare presentations is pulmonary-renal syndrome.Case Presentation: We report a case of a 64-year-old African American woman, who presented to our emergency room with shortness of breath, orthopnea, paroxysmal nocturnal dyspnea, leg swelling, fatigue, loss of appetite, cough with clear sputum, and lightheadedness. On admission, she developed acute hypoxic respiratory failure requiring intubation and acute renal failure requiring hemodialysis. A serologic workup was positive for antineutrophil cytoplasmic antibody, antinuclear antibody, anti-histone, anti-cardiolipin IgM, and anti-double-stranded DNA antibodies. A renal biopsy was done due to persistent deterioration in kidney function and demonstrated classic crescentic (pauci-immune) glomerulonephritis. Hydralazine was empirically discontinued early in the admission and she was started on corticosteroids and cyclophosphamide following biopsy results. She was clinically stable but remained dependent on hemodialysis after discharge.Conclusion: Hydralazine-induced antineutrophil cytoplasmic antibody-associated vasculitis with pulmonary-renal syndrome is a rare occurrence. In the setting of hydralazine use, multiple positive antigens, and multisystem involvement, clinicians should consider this rare condition requiring prompt cessation of offending drug, early evaluation with biopsy, and contemplate empiric immunosuppressive therapy while biopsy confirmation is pending. [ABSTRACT FROM AUTHOR]

Whilst they are rare paediatric rheumatological emergencies can be life-threatening. They may occur in a patient with or without a known underlying rheumatological diagnosis. The signs and symptoms of a rheumatological emergency may be subtle and non-specific and can easily be missed, especially in a patient without a known underlying connective tissue disease or vasculitis. Progression can be rapid and without prompt recognition and treatment such emergencies can be catastrophic. This paper discusses the presenting features and the approach to management of several rheumatological emergencies including Macrophage Activation Syndrome (MAS), Pulmonary-Renal Syndrome, neonatal heart block, central nervous system (CNS) vasculitis and Catastrophic Antiphospholipid Syndrome (CAPS). Awareness of these conditions facilitates prompt recognition, guides appropriate investigations and ensures timely initiation of treatment. This is essential to help prevent avoidable morbidity and mortality. [ABSTRACT FROM AUTHOR]

In this study, the cases of a patient with Goodpasture syndrome (also referred to as anti-glomerular basement membrane disease) and a patient with the morphological features of Goodpasture syndrome or Goodpasture-like syndrome found in a histopathological examination are described. In both cases the course of the disease was atypical, with a massive respiratory tract hemorrhage being the most significant symptom. Accordingly, the presented patients were treated surgically. Furthermore, the paper described diagnostic difficulties in identifying the cause of the hemorrhage. Finally, the importance of histopathological examination in the diagnosis of Goodpasture syndrome and the Goodpasture-like syndrome with atypical course is emphasized. [ABSTRACT FROM AUTHOR]

Diffuse alveolar hemorrhage (DAH) is a rare but potentially life-threatening condition characterized by bleeding into the alveolar spaces of the lungs. DAH can occur due to a wide range of etiologies including autoimmune diseases, infections, drugs, and malignancies. The clinical presentation is variable and may include cough, dyspnea, fever, and hemoptysis. Diagnosis is often challenging due to the nonspecific symptoms and a lack of definitive diagnostic criteria. Treatment is primarily aimed at addressing the underlying cause and providing supportive care., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Bardarov et al.)

El síndrome pulmón-riñón rara vez ha sido reportado como cuadro clínico de presentación de vasculitis causada por el consumo de cocaína contaminada con levamisol. Se reporta el caso de un paciente con este cuadro clínico y se señalan las dificultades que se presentaron durante el abordaje diagnóstico y terapéutico. Pulmonary-renal syndrome has rarely been reported as the clinical presentation of vasculitis caused by the consumption of cocaine adulterated with levamisole. We report the case of a patient in whom we detected the clinical manifestations and indicate the difficulties that arose in relation to the diagnostic and therapeutic approach. [ABSTRACT FROM AUTHOR]

Background: Anti-glomerular basement membrane (anti-GBM) disease is characterized by circulating IgG glomerular basement membrane antibodies and is clinically expressed as a rapidly progressive crescentic glomerulonephritis (GN), with 30-60% of patients also developing pulmonary hemorrhage. Classically, the renal biopsy shows glomerular crescent formation, bright linear staining of glomerular basement membranes (GBM) for IgG on direct immunofluorescence (IF), and the serologic presence of circulating anti-GBM antibodies. Recently, patients with linear IgG IF staining, undetectable circulating anti-GBM antibodies and glomerular changes atypical for anti-GBM disease have been described as "atypical anti-GBM disease", with a distinctly more benign clinical course than typical anti-GBM disease. We present a case report of a patient with negative anti-GBM serology but positive linear IgG staining by IF, severe diffuse crescentic and endocapillary proliferative glomerulonephritis, and renal failure, complicated by severe pulmonary hemorrhage after immunosuppression, likely due to cytomegalovirus (CMV) pneumonitis.Case Presentation: A 24-year-old man was admitted to hospital with hemoptysis and renal failure. Investigations for anti-GBM serology by addressable laser bead immunoassay (ALBIA) was negative for anti-GBM antibodies. Renal biopsy showed diffuse endocapillary proliferative glomerulonephritis with membranoproliferative features and diffuse circumferential crescents. Direct IF showed strong linear staining for IgG along GBMs. The patient's hemoptysis improved with immunosuppression, but 1 month later he was readmitted with gross hemoptysis, which was refractory to further cyclophosphamide, plasma exchange and rituximab. Bronchoalveolar lavage (BAL) and blood work confirmed CMV pneumonitis, and the patient's hemoptysis resolved with ganciclovir, though he became dialysis dependent.Conclusions: This case demonstrates an atypical presentation of anti-GBM disease with both crescents and endocapillary hypercellularity and negative serology. The patient is dialysis dependent, unlike most previously described patients with atypical anti-GBM disease. The course was complicated by CMV pneumonitis, which contributed to the severity of the pulmonary manifestations and added diagnostic difficulty. [ABSTRACT FROM AUTHOR]

Pulmonary-Renal Syndrome (PRS) is a rare clinical feature characterized by the simultaneous presence of diffuse alveolar hemorrhage and glomerulonephritis. PRS is a serious and sometimes life-threatening complication of several conditions, including systemic autoimmune diseases. The diagnosis is based upon clinical, serological, radiological and histological findings. Early treatment greatly affects on prognosis, improving mortality and long-term sequelae. In our case report, microscopic poliangiitis was diagnosed and the patient showed a favorable clinical course after starting corticosteroid and cyclophosphamide therapy [ABSTRACT FROM AUTHOR]

Antiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of cases may present with pulmonary disease in isolation. Serological testing for anti-GBM antibodies may facilitate rapid diagnosis, though renal biopsy is often required to confirm the presence of necrotizing or crescentic GN and linear deposition of autoantibody on the glomerular basement membrane. Alveolar hemorrhage may be evident clinically, or detected on imaging, pulmonary function testing, or bronchoscopy. Prompt treatment with plasmapheresis, cyclophosphamide, and steroids is usually indicated to remove pathogenic autoantibodies, to prevent their ongoing production, and to ameliorate end-organ inflammation. Alveolar hemorrhage is usually responsive to this treatment, and long-term respiratory sequelae are uncommon. Renal prognosis is more variable, though with aggressive treatment, independent renal function is maintained at 1 year in more than 80% of patients not requiring renal replacement therapy at presentation. Relapse in uncommon in anti-GBM disease, unless there is a concomitant antineutrophil cytoplasm antibody (present in 30-40%), in which case maintenance immunosuppression is recommended. [ABSTRACT FROM AUTHOR]

Abstract Scleroderma associated Pulmonary–Renal Syndrome is a rare but severe complication with a poor prognosis and high mortality. A high index of suspicion is needed for early recognition of this potential complication in patients with systemic sclerosis and institution of appropriate treatment. With more data showing an increased association between scleroderma and malignancy, a heightened vigilance should also be exercised in patients with malignancy and scleroderma-like presentation. We report of a case rapid onset systemic sclerosis complicated by acute renal failure and diffuse alveolar hemorrhage in a woman with stage IIB right breast cancer and elevated RNA Polymerase III IgG Ab. To our knowledge, this the first case of a patient with breast cancer associated with systemic sclerosis and pulmonary-renal syndrome. [ABSTRACT FROM AUTHOR]

Copyright of Review of Medicine / Przeglad Lekarski is the property of Wydawnictwo Przegld Lekarski / Publisher Medicine Review and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Review of Medicine / Przeglad Lekarski is the property of Wydawnictwo Przegld Lekarski / Publisher Medicine Review and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Granulomatosis with polyangitis (GPA) is one of systemic connective tissue diseases. Necrotizing vasculitis affects small- and medium-sized vessels. Antineutrophil cytoplasmic antibodies directed against the neutrophil serine protease proteinase-3 (cANCA, PR3-ANCA) have been identified in approximately 80–95% of patients with GPA. In typical cases upper and lower respiratory tract and kidneys are involved. However GPA produce a broad spectrum of clinical symptomes, that includes: ocular and ear involvement, peripheral and central nervous system manifestations, heart and digestive tract involvement. The diffuse alveolar hemorrhage and pulmonary-renal syndrome may also occur. The diagnosis of granulomatosis with polyangiitis may cause many difficulties, specially in cases with limited organ involvement, with untypical spectrum of clinical features and in ANCA-negative patients. We attempt to present differential diagnosis of GPA in this review article.

Cryoglobulinemia (CG) is defined as the presence of abnormal immunoglobulins (Igs)that precipitate at low temperatures and dissolve upon warming. The manifestations in type I cryoglobulinemia are often related to intravascular obstruction which include skin, joint, renal and neurological involvement. We report a rare case of type 1 CG that presented with diffuse alveolar hemorrhage. Following extensive workup, the patient was found to have membranoproliferative glomerulonephritis secondary to type 1 CG in the setting of marginal B cell lymphoma. He was started on an aggressive regimen targeting underlying lymphoma. Key to managing this condition was multidisciplinary approach towards the diagnosis and management of this otherwise challenging case. [ABSTRACT FROM AUTHOR]

Introduction and Importance: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare co-occurrence with systemic sclerosis, in around 2.5-9% of patients. The clinical manifestations and prognosis of vasculitis in systemic sclerosis depend on organ involvement. It presented with rapidly progressive acute renal failure without malignant hypertension, and with pitting hand and foot ulcers get along with purpuric vasculitis in some cases reports. Reports had found that survival in those with pulmonary-renal syndrome is poor. However, high-dose corticosteroids and cyclophosphamide increase the survival percent in those patients., Case Presentation: An 81-year-old female was admitted for newly diagnosed acute renal failure and highly elevated C-reactive protein levels. She was diagnosed with systemic sclerosis 8 years previously, with a 3-year history of interstitial lung disease, and a 2-year history of pulmonary hypertension. Treatment included home oxygen on demand, prednisone 5 mg/day, and azathioprine 75 mg daily. On physical examination, she had sclerodactyly, both extremities ulcers, severe livedo reticularis, and hyperpigmented papules on her hand and feet. Laboratory findings included a markedly positive MPO (p-ANCA), and anti-Scl-70. She was treated with pulse methylprednisolone without any improvement. After a day, she developed anuria and became comatose. Then, she developed cardiac arrest, leading to death., Clinical Discussion: The presence of ANCA in systemic sclerosis patients ranges from 2.5 to 9% of systemic sclerosis patients. It presented with rapidly progressive acute renal failure without malignant hypertension, and with pitting hand and foot ulcers. The treatment with high-dose corticosteroids and cyclophosphamide is benefit. Survival in those with pulmonary-renal syndrome is poor., Conclusion: The presence of ANCA-associated vasculitis is rarely reported with scleroderma. It occurs most commonly in women with limited or Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia (CREST) variants of scleroderma, as well as those with overlap features. Severe manifestations including pulmonary-renal syndrome and death may occur., Competing Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.)

Wegener's granulomatosis (WG) is a systemic vasculitis characterized by necrotizing granulomatous inflammation of small and medium-sized blood vessels and upper respiratory tract, lungs and kidneys [1]. WG is traditionally considered to be a a rare disease the prevalence of which is 25-60 people per 1 million of population; however, the last 30 years have seen a 4-fold increase in the incidence of the disease [2]. 90% of patients die within the first 2 years of the onset of the disease without treatment. Timely diagnosis of WG is therefore important because the disease if often hard to diagnose in the beginning due to atypical oligosymptomatic flow.

Copyright of Revista Médica de Rosario is the property of Circulo Medico de Rosario and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)