Pauci-Immune Pulmonary Capillaritis Research Articles

Isolated pauci-immune pulmonary capillaritis associated with an extensive bilateral hemothorax and pleuro-pericarditis

Isolated pauci-immune pulmonary capillaritis associated with an extensive bilateral hemothorax and pleuro-pericarditis

A RARE CASE OF PAUCI-IMMUNE PULMONARY CAPILLARITIS WITH PERIPHERAL EOSINOPHILIA

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a syndrome classically presenting with hemoptysis, diffuse alveolar infiltrates, and acute respiratory failure. It is usually associated with systemic vasculitis and auto-antibodies. Idiopathic pauci-immune pulmonary capillaritis (IPPC) is a rare form of isolated DAH that presents without other clinical features of systemic vasculitis or circulating auto-antibodies. We present a patient with DAH from IPPC, who was also found to have peripheral eosinophilia. CASE PRESENTATION: 76-year-old female presented with subacute complaint of generalized weakness and confusion. Her past medical history was significant for multiple sclerosis treated with rituximab. Initial vital signs were afebrile, BP 126/66, HR 78, RR 22, saturating 95% on 1 liter nasal cannula. Physical examination findings were significant for a markedly obese woman, decreased breath sounds and crackles noted in both lung bases, 3+ edema in both lower extremities. Laboratory workup revealed a WBC count of 7.9/µL with 30% eosinophils, hemoglobin 10 g/dL and platelets 84K. Urinalysis was consistent with urinary tract infection (UTI). Chest x-ray revealed a right basilar infiltrate and effusion, small left basilar infiltrate, pulmonary venous congestion and mild cardiomegaly. She was started on an antibiotic for UTI and a diuretic for preliminary diagnosis of heart failure. Despite diuretics, her oxygen requirements continued to increase. CT chest showed bilateral infiltrates and extensive bilateral ground glass changes. Infectious and auto-immune workup were negative. Bronchoscopy showed progressive bloody return on sequential lavage consistent with DAH. Cytology of the bronchial lavage (BAL) fluid was negative for malignancy and did not show any organisms or eosinophilia. She was treated with steroids and plasmapheresis. DISCUSSION: The differential diagnosis for DAH is broad, but mostly consists of various underlying systemic diseases, none of which our patient manifested with despite extensive work-up. The remaining practical diagnosis of exclusion was IPPC. Furthermore, her presentation included eosinophilia, although she did not appear to manifest with any of the common differentials for peripheral eosinophilia. Her presentation was not consistent with disorders such as acute eosinophilic pneumonia or acute interstitial pneumonia as her BAL did not reveal eosinophilia or neutrophilia, respectively. This may have been confounded by steroid treatment. It remains possible that her initial peripheral eosinophilia was due to an unrelated process from her pulmonary disease. CONCLUSIONS: Our patient manifested with pauci-immune pulmonary capillaritis. Our case also demonstrates concurrent peripheral eosinophilia which has not been previously described in this already rare disease. Further studies are needed to understand the pathogenesis of IPPC. REFERENCE #1: Park MS. Diffuse alveolar hemorrhage. Tuberc Respir Dis (Seoul). 2013;74(4):151-162. doi:10.4046/trd.2013.74.4.151 REFERENCE #2: Jason S. Oh, Uni Wong, Divyansh Bajaj, Stella E. Hines, "Isolated Pauci-Immune Pulmonary Capillaritis Associated with Hydrocarbon Inhalation and Marijuana Smoking: An Unusual Case of Severe Hypoxemia", Case Reports in Pulmonology, vol. 2020, Article ID 1264859, 5 pages, 2020. https://doi.org/10.1155/2020/1264859 DISCLOSURES: No relevant relationships by Joseph Bahgat, source=Web Response No relevant relationships by Frantz Hastrup, source=Web Response

Alveolar Hemorrhage in Vasculitis (Primary and Secondary).

Defined by the accumulation of red blood cells into the alveolar space, diffuse alveolar hemorrhage (DAH) is a severe and potentially fatal medical condition requiring careful attention. In contrast to simple extravasation of erythrocytes facilitated by impaired hemostasis or hemodynamic causes, DAH in vasculitis is due to capillaritis, that is, inflammation of capillaries. Dyspnea, hemoptysis, chest infiltrates, and abrupt fall of blood hemoglobin level represent the cardinal features of DAH; yet, hemoptysis is lacking in one-third of cases. Bronchoalveolar lavage, retrieving bright red fluid, is the best diagnostic clue, also excluding infection and other causes of hemoptysis. Although not recommended, lung biopsy is the gold standard for the diagnosis of DAH and pulmonary capillaritis. Pulmonary capillaritis may be primary as in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis or secondary to drugs (especially antithyroid drugs such as propylthiouracil), infections, connective tissue diseases especially systemic lupus erythematosus, or other small vessel vasculitides. Newer toxic causes of drugs of abuse may be difficult to diagnose. Granulomatosis with polyangiitis and microscopic polyangiitis are the most common causes of capillaritis and DAH, whereas DAH is extremely rare in eosinophilic granulomatosis with polyangiitis. When pulmonary capillaritis is not secondary to underlying systemic vasculitis, idiopathic pauci-immune pulmonary capillaritis may be considered, with or without ANCA. Supportive treatment strategy is mandatory in all cases of DAH. Mechanical ventilation and extracorporeal membrane oxygenation may be used in severe cases. Early identification and removal of the putative drug is crucial in drug-induced vasculitis/DAH and may obviate the need for immunosuppressive therapy. High-dose corticosteroids, intravenous cyclophosphamide, and recently rituximab are the mainstay of treatment in vasculitis. Plasma exchange is recommended in anti-glomerular basement membrane disease and in severe DAH associated with systemic lupus erythematosus and is used in selected cases in ANCA-associated vasculitis.

A Rare Case of Isolated Pauciimmune Pulmonary Capillaritis in a Child

A Rare Case of Isolated Pauciimmune Pulmonary Capillaritis in a Child

Pulmonary vasculitis and pulmonary hemorrhage

Pulmonary vasculitis is a rare disease that typically shows inflammation in pulmonary vessel walls and necrosis.1 The disease is usually immune mediated, common in young patients, triggered by many factors, and with wide clinical and radiologic presentations. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAVs) is the main focus in most literature and includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), Churg–Strauss syndrome (CSS), idiopathic pauci immune pulmonary capillaritis (IPIPC), secondary to systemic lupus erythematosus, and other types like anti-glomerular basement membrane disease, drug-induced vasculitis after cannabis and glue inhalation.2 Diagnosis is usually established after careful history taking clinical, radiologic, and laboratory evidence; bilateral lung infiltrates with or without pulmonary hemorrhages and peripheral distribution, positive ANCA-P component, proteinase-3 ELISA test, anti-glomerular basement membrane antibody, and other antibodies associated with immunologic disease like systemic lupus erythematosus. Sometimes, lung biopsy may be needed but because of the low yield of bronchoscopic biopsies and the need for open lung biopsy, this tool is rarely used specially in critically ill patients. Treatment is by the use of steroids and cytotoxic medications like cyclophosphamide and mycophenolate mofetil. In refractory cases, plasmapheresis or the monoclonal antibody rituximab may be used. Pulmonary hemorrhage is a common manifestation in vasculitic lung syndromes; however, many other disease and toxins can trigger pulmonary hemorrhage, which can be life threatening.3 The diagnosis of pulmonary hemorrhage is generally considered based on clinical and radiological findings and is characterized by acute bilateral patchy lung infiltrates more centrally located but may be diffuse. Sequential aliquots of bronchoalveolar lavage yield progressively bloodier return and help in diagnosis.4 Treatment depends largely on the cause. Extracorporeal membrane oxygenation (ECMO) is a life support modality that can be used in severe cases of pulmonary vasculitis and hemorrhage with great caution and careful assessment of risk of bleeding with anticoagulation and benefit of use as life-saving support. With newer ECMO circuits used (either heparin- or bioline-coated circuits), several days of heparin-free ECMO runs may be successful. In some reported cases, citrate was used as an anticoagulant.5 The ECMO modality shall be carefully chosen especially in the presence of pulmonary hypertension, which may affect the veno-venous (VV) ECMO flow, and hence, echocardiographic assessment before initiation is mandatory to avoid low-efficiency runs. In cases with severe pulmonary hypertension, veno-arterial (VA) ECMO would be more suitable. Institution of treatment using steroids and cytotoxic drugs in addition to plasmapheresis is warranted upon suspected diagnosis. The rapid initiation of treatment may be the key point of survival in such cases. There are some case reports where plasmapheresis and immunosuppressive therapies were used based on clinical and radiographic data alone5 with successful outcome. There is paucity of data regarding drug doses of immunosuppressive drugs used during the ECMO run, and the duration of treatment was variable. Most of the cases reported started with 1 g of methylprednisolone for 3 days followed by either plasmapheresis and cyclophosphamide or cyclophosphamide and rituximab.6 The plasmapheresis circuit installation needs to be addressed whether to be incorporated into the ECMO circuit7 or not.

A Rare Case of Isolated Pauciimmune Pulmonary Capillaritis (IPPC): Managing Recurrent Alveolar Hemorrhage and Concomitant Pulmonary Embolism

SESSION TITLE: Student/Resident Case Report Poster - Diffuse Lung Disease SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a clinicopathological syndrome that can rapidly progress into life threatening respiratory failure. Most cases are associated with systemic vasculitides, and management is aimed at diagnosis and treatment of the underlying cause. Less is known about the management of IPPC. CASE PRESENTATION: A 74-year-old woman first presented to our institution in 2010 with dyspnea and hemoptysis. Chest CT showed diffuse infiltrates (Image 1), and video assisted thoracoscopic lung biopsy revealed pulmonary hemorrhage with focal neutrophilic capillaritis. Extensive work-up (including ANCA panel, ANA, rheumatoid factor, anti-dsDNA, celiac serologies, anti-GBM, anti-cardiolipin, cryoglobulins, vWF) was unrevealing for an underlying cause, suggesting the diagnosis of IPPC. Prednisone and cyclophosphamide were initiated, resulting in symptom improvement, but development of atypical bladder cytology necessitated discontinuation of cyclophosphamide. She was tried on azathioprine then later mycophenolate, but had recurrent DAH after attempts at dose reduction. Her case was further complicated by out of hospital thrombotic events. In 2011, she represented with bilateral PE (Image 2), and an IVC filter was placed as an alternative to anticoagulation which posed a life-threatening bleeding risk. Aspirin 81mg daily was initiated which was changed to every-other-day dosing when she had another DAH flare. Four years later, she had a second out of hospital PE. She was treated with heparin drip and discharged on rivaroxaban, but unfortunately experienced recurrence of DAH. Rivaroxaban was discontinued, high dose prednisone was initiated, and her IVC filter was replaced. She also completed a four-week course of weekly rituximab, and restarted Aspirin 81mg daily. On this therapy she has remained free of both alveolar hemorrhage and pulmonary thrombosis. DISCUSSION: Only a handful of cases on IPPC have been reported in the literature, with a single case report of successful remission with rituximab. Thus far, an association with IPPC and hypercoagulable disease has not been reported. The exact etiology of our patient’s PE remains unclear. Extensive work up revealed no predisposition for clotting, and ultrasound showed no DVT. Acute thromboembolism is increasingly recognized as a complication of many systemic vasculitides, and it is possible that her disease activity triggered the hypercoagulable state responsible for her thrombotic events. CONCLUSIONS: Both alveolar hemorrhage and de novo clot formation are possible complications of IPPC. Development of thromboembolism in the setting of DAH presents a significant management dilemma. This case sheds light on how both life-threatening complications can be managed by carefully balancing these risks and suppressing the underlying capillaritis. Reference #1: Thompson G, Specks U, Cartin-Ceba R. Isolated pauciimmune pulmonary capillaritis successfully treated with rituximab. CHEST Journal. 2015;147(4):e134-e136 DISCLOSURE: The following authors have nothing to disclose: Natsu Fukui, Eric Libre, John Paul Verderese, Christopher King No Product/Research Disclosure Information

A rare case of isolated pauci-immune pulmonary capillaritis.

A rare case of isolated pauci-immune pulmonary capillaritis.

Diffuse alveolar hemorrhage resulting from Pauci-immune pulmonary capillaritis

A 27 year-old female patient, cocaine user, presenting hemoptysis and progressive dyspnea with onset 48 hours prior to hospital admission, without any other signs or symptoms. Serum tests for infectious diseases, collagen disorders and vasculitis were negative. Urinalysis was normal. Computed tomography of the chest showed diffuse alveolar infiltrate, affecting mainly the lower left lobe. A thoracoscopic lung biopsy was performed to clarify the diagnosis. The histopathological findings showed capillaritis and diffuse intra-alveolar hemorrhage. Treated with steroid and cyclophosphamide pulse therapy, a good clinical and radiographical response was obtained. The recently described pauci-immune pulmonary capillaritis is characterized by the presence of isolated pulmonary capillaritis and negative serum testing for auto-immune diseases.