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Case report - Introduction The COVID-19 pandemic led to drastic changes for some patients on warfarin for venous thromboembolic (VTE) disease and atrial fibrillation. Warfarin monitoring necessitates frequent interaction with healthcare workers, which is sufficiently risky for COVID-19 transmission. As a result, selected patients were swapped over to novel oral anticoagulants (NOACs). Our patient was changed without investigating for antiphospholipid syndrome (APLS); it later transpired he was triple antibody positive. He presented in a crisis and we describe his narrative. Patients on warfarin due to presumed unprovoked venous thromboembolic disease should not be swapped to NOACs without completing, or checking, previous antiphospholipid antibody testing. Case report - Case description A 73-year-old gentleman presented locally in August 2020 with erythema over the anterolateral surface of his left leg. He was initially treated with antibiotics for presumed cellulitis. Within a few days this lesion became necrotic and rapidly spread. At this point, he was transferred to a tertiary rheumatology centre. Within days to weeks, he developed several necrotic lesions affecting his trunk and limbs, with facial sparing noted. Approximately 30—35% of his whole-body surface became involved. He soon developed an oxygen requirement, with CTPA demonstrating lymphocytic interstitial pneumonitis without evidence of pulmonary emboli (PE). Throughout his admission, he had several other pathologies such as hyponatraemia that required level 2 care and severe non-infectious diarrhoea. Skin biopsy identified thrombotic vasculopathy. Serology confirmed triple positive antiphospholipid antibody status and a dsDNA titre of > 400 iU/mL. This was the first-time serology had been undertaken despite a history of three deep vein thrombosis (DVT) episodes and two PE incidents. He had no history of SLE symptoms. His initial management for vasculitis secondary to APLS at the point of limited necrosis consisted of IV methylprednisolone followed by rituximab and PO prednisolone. While there was some delay in the progression of his disease, new areas of necrosis arose, leading to the patient receiving cyclophosphamide. Low molecular weight heparin was used for anticoagulation. This gentleman later developed proteinuria and neurological symptoms, fulfilling the criteria for catastrophic antiphospholipid syndrome. He received plasma exchange, without an improvement. He developed complications from his disease and treatment, including poor wound healing. It became apparent his condition would not improve and active treatments were stopped. He passed away 6 weeks after initial presentation. Prior to his admission to hospital, his warfarin was swapped to a NOAC. This is thought to have been the trigger behind catastrophic thrombosis. Case report - Discussion After excluding other conditions such as necrotising fasciitis, this gentleman was rapidly started on IV methylprednisolone to halt any further progression. This is because glucocorticoids have the greatest evidence base for managing this poorly understood acute disease manifestation. After this failed to manage his condition, he was given a further immunosuppressive agent in the form of rituximab. This was used after his serology confirmed triple antibody status. It was hoped this would stop any further immunological mediated disease progression. Oral prednisolone was started at 40 mg at this stage and kept under review with a tapering schedule. Cyclophosphamide was given within a few days of rituximab, with hope of a quicker onset of action. A careful MDT decision was made on these drug choices, particularly regarding their combined use and appreciating their side effect profiles. Cyclophosphamide has evidence behind its use, especially for those with APLS associated with lupus. While he did not develop any infections related to treatment, his condition progressed. Case reports suggest that plasma exchange can be useful in the management of catastrophic antiphospholipid syndrome, so the team recommended this. Consent at this stage became tricky due to his altered mental status, but it was felt he did demonstrate capacity for this specific decision. As his condition did not improve after this level of immunosuppression, the team reached the decision that no other treatments would likely change the outcome. He remained on oral steroids for the remainder of his admission. The other management facet of APLS crises pertains to anticoagulation. Low molecular weight heparin was recommended by the haematologists. His NOAC was stopped after the diagnosis was confirmed. Warfarin was restarted later in his admission given he had been well on this for years. Case report - Key learning points This fascinating case exemplifies the importance of completing an antiphospholipid antibody screen for patients who present with unprovoked venous thromboembolic disease. NOACs are commonly used anticoagulant medications. Several case reports have demonstrated that patients with antiphospholipid syndrome experience breakthrough thromboembolic events when treated with NOACs. The highest risk is associated with history of arterial thrombosis and those with triple positive antibody status. Three clinical trials have either been completed or are in the process of investigating whether NOACs sufficiently prevent thromboembolic disease in these patients. The TRAPS study compared rivaroxaban to warfarin in those with triple antibody positive antiphospholipid syndrome. The study was terminated early given that higher adverse events were observed in the rivaroxaban arm (19%, n = 11/59) versus warfarinised patients (3%, n = 2/61). The RAPS study found no difference in thromboembolic risk and results from the ASTRO-APS study looking into apixaban are awaited. There is insufficient evidence to suggest that NOACs prevent VTE in a similar fashion to warfarin, so many still advocate the use of warfarin. The optimal immune management of this acute complication is not well elucidated, with a shortfall in mechanistic pathological understanding. The conference will generate discussion on this subject matter in detail. During the COVID-19 pandemic, it has been observed for patients to change anticoagulation from warfarin to NOACs. Given NOACs do not require monitoring, this medication change reduces the number of interactions patients have with healthcare services. We postulate this change triggered the crisis in our patient, where we suggest continuation of warfarin would have been ideal. This is due to the history of several unprovoked thromboembolic events without a prior antiphospholipid screen being completed. Dissemination of learning points from this case are imperative to ensure decision-making encompasses patients who may have undiagnosed antiphospholipid syndrome.

Background An increase has been described throughout the years in the frequency of various uncommon diseases in people living with human immunodeficiency virus (HIV). Particularly late presenters are associated with a significant risk not only for acquired immune deficiency syndrome (AIDS)-defining conditions but also for non AIDS-defining diseases which aggravate the prognosis of patients. Lymphoid interstitial pneumonitis (LIP) is one of these conditions described more often after the onset of HIV epidemic. LIP is a benign polyclonal lymphoproliferative disorder of the lung with not well characterized clinical and radiographic findings. Case presentation We report the diagnostic approach and clinical progress of a newly diagnosed late presenter of HIV infection with respiratory problems in our HIV unit. The findings of computed tomography indicated the diagnosis of HIV-associated LIP, although this condition is mainly described in a normal range of CD4 cell count. Conclusion This case presentation highlights the importance of timely diagnosis and initiation of antiretroviral therapy. The increase of CD4 cell count and viral suppression may improve the symptoms of LIP.

Case report - Introduction Sjögren’s syndrome (SS) is a chronic autoimmune inflammatory condition characterised by lymphocytic infiltration of predominantly exocrine glands as well as other organs involving more commonly in skin, lungs, and neurological system. It is associated with increased risk of both benign and malignant lymphoproliferative disorders. Case report - Case description A 29-year-old woman with 5-year history of Primary SS (ANA, Ro/La positive) was reviewed in a routine follow up. She has mild dry eyes, intermittent vasculitic rash around the ankles, occasional pleuritic chest pain but no cough or dyspnoea initially. She used artificial tears but took no regular mediations. Oxygen saturation at rest was 99% and BP 127/70. Heart sounds were normal, and chest was clear. Blood results showed alanine transaminase (ALT) had risen from 50 (normal 5-45) to 145 over 6 months. Viral screen (hepatitis, HIV, CMV, EBV) and liver ANA panel were negative. C4 was low 0.09 (n 0.15-0.55), C3 normal and DsDNA antibodies 1 (n 0-15.1). Ig G was high, fluctuating between 27 - 33 (n 7.67-15.9). ESR had been mildly elevated at 26 (n 0-19). CRP had always been within normal limits. Ultrasound abdomen showed enlarged spleen (15 cm). CT chest abdomen and pelvis showed multifocal groundless opacification affecting mid to upper zones, bilateral cyst formation, solid nodularity, and mild septal thickening with no pleural effusion. There were multiple prominent bilateral axillary and mediastinal lymph nodes measuring upto15 mm. The spleen measured 15.4 cm. Six months later, she developed mild dyspnoea on exertion. Examination revealed fine basal crackles. Oxygen saturation remained 99% at rest. TLco was mildly reduced and Kco in lower limit of normal. Echocardiogram was normal. Ultrasound guided core biopsy of axillary lymph node showed benign reactive features with follicular hyperplasia and plasmacytosis. The diagnosis of lymphocytic interstitial pneumonitis was made. She was started on a reducing dose of Prednisolone 30 mg and Mycophenolate stepwise increment to 1 gm BD. Chest pain resolved shortly after starting glucocorticoid therapy and exercise tolerance improved. Liver enzyme normalised. Case report - Discussion The diagnosis of lymphocytic interstitial pneumonia (LIP) in this patient was incidental from the retrospective investigation of the persistently abnormal unexplained liver function test. Splenomegaly is a recognised manifestation of active systemic SS. The main differential diagnoses are lymphoma and other types of interstitial lung diseases (ILD). Her presentation was insidious and there were no constitutional symptoms. Her ANA, Ro, La and Rheumatoid factor were strongly positive. She has hypergammaglobulinemia which is commonly associated with LIP. Histological findings were typical reactive changes with characteristic polymorphic plasmacytosis. In the absence of malignant lymphoid cells and radiological features of ground glass changes and bilateral cysts formation suggested that the most likely diagnosis was LIP secondary to primary SS. The management strategies vary for different types of ILD based on their etiological conditions. There are no specific guidelines for the treatment of LIP. For initial acute stage, glucocorticoid therapy is usually prescribed for symptomatic improvement. For later stages and cysts, immunosuppressants are used and depending on the severity, azathioprine, cyclosporin, rituximab and cyclophosphamide have been used. Median survival for LIP is 11.2 years. Increased risk of secondary infection because of immunosuppressive therapy is common and there are recommendations for atypical chest infection prophylaxis. Case report - Key learning points Interstitial lung disease is commonly associated with SS and predicts a higher mortality rate. Glucocorticoid is the main stay of treatment for ILD acute stage with active inflammatory changes. For refractory cases and chronic cystic stages on radiological findings, further immunosuppression therapy may be required. Standardised treatment guidelines are not available. Secondary chest infections may be seen in those with cystic disease and on immunosuppression. Close monitoring and MDT approach is required in the management of LIP. LIP may transform into lymphoma or co-present with lymphoma associated with SS.

ResumenAntecedentesPoco se sabe sobre la neumonitis intersticial linfoidea (NIL) en niños con infección por el virus de inmunodeficiencia humana-1 (VIH-1).ObjetivoDescribir las características clínicas y patológicas de NIL en niños infectados por VIH-1 en un centro de referencia para VIH pediátrico en Cali (Colombia).MétodosSe llevó a cabo una descripción de serie de casos de NIL basados en revisión retrospectiva de historias clínicas de todos los casos de niños con enfermedad pulmonar crónica y LIP confirmada por biopsia entre los años 2001 y 2012.Resultados y conclusionesDiez de 12 casos con NIL fueron confirmados por biopsia pulmonar. Se observó una respuesta clínica y de función respiratoria luego del tratamiento con prednisona, excepto en un caso que presentó tos persistente. No se encontraron casos de tuberculosis pulmonar (TP) en nuestra serie y el papel de la biopsia pulmonar fue crítico para alcanzar un diagnóstico preciso.AbstractBackgroundLittle is known about Lymphoid Interstitial Pneumonitis (LIP) in children with HIV infection.AimsTo describe the clinical and pathological characteristics of LIP in infected children in a referral center for pediatric HIV in Cali (Colombia).MethodsCase series based on retrospective analysis of clinical charts among HIV-infected children with chronic lung disease and lung-biopsy proven LIP between the years 2001 and 2012.Results and conclusions10 of 12 cases of LIP were confirmed by lung biopsy. Significant clinical and respiratory functional improvement was obtained in all cases after prednisone therapy, excepting one child who presented persistent cough. No case of pulmonary TB was detected in our cohort. Lung biopsy was critical to obtain an accurate diagnosis.

Resume Les manifestations pulmonaires non infectieuses (MPNI) sont meconnues chez les patients ayant un deficit immunitaire commun variable (DICV), les complications les plus frequentes etant infectieuses. Nous rapportons une serie de 11 patients ayant presente une MPNI, suivis dans le service de pneumologie de deux hopitaux parisiens, de 1990 a 2008. Le taux moyen de gammaglobulines seriques etait de 3,46 g/dL. Les MPNI etaient : fistule arterioveineuse pulmonaire : trois cas ; pneumopathies interstitielles diffuses : trois cas ; asthmes : deux cas ; adenopathies mediastinales avec aspect d’hyperplasie lymphoide : quatre cas ; emphyseme : un cas ; mesotheliome : un cas. Les MPNI sont frequentes et variees chez les patients ayant un DICV et doivent etre recherchees systematiquement.

Antecedentes : Poco se sabe sobre la neumonitis intersticial linfoidea (NIL) en niños con infección por el virus de inmunodeficiencia humana-1 (VIH-1) Objetivos: Describir las características clínicas y patológicas de NIL en niños infectados por VIH-1 en un centro de referencia para VIH pediátrico en Cali (Colombia). Métodos: Se llevó a cabo una descripción de serie de casos de NIL basados en revisión retrospectiva de historias clínicas de todos los casos de niños con enfermedad pulmonar crónica y LIP confirmada por biopsia entre los años 2001 y 2012. Resultados y conclusiones: Diez de 12 casos con NIL fueron confirmados por biopsia pulmonar. Se observó una respuesta clínica y de función respiratoria luego del tratamiento con prednisona, excepto en un caso que presentó tos persistente. No se encontraron casos de tuberculosis pulmonar (TP) en nuestra serie y el papel de la biopsia pulmonar fue crítico para alcanzar un diagnóstico preciso. Background: Little is known about Lymphoid Interstitial Pneumonitis (LIP) in children with HIV infection. Aims: To describe the clinical and pathological characteristics of LIP in infected children in a referral center for pediatric HIV in Cali (Colombia). Methods: Case series based on retrospective analysis of clinical charts among HIV-infected children with chronic lung disease and lung-biopsy proven LIP between the years 2001 and 2012. Results and conclusions: 10 of 12 cases of LIP were confirmed by lung biopsy. Significant clinical and respiratory functional improvement was obtained in all cases after prednisone therapy, excepting one child who presented persistent cough. No case of pulmonary TB was detected in our cohort. Lung biopsy was critical to obtain an accurate diagnosis.

Undiagnosed or untreated human immunodeficiency virus infection can lead to devastating complications. We present a case of a 41-year-old woman who was found to have HIV-related lymphoid interstitial pneumonitis. LIP is uncommon, and its presentation can be quite similar to that of other chronic lung conditions. This case illustrates one of the possible protean manifestations of untreated HIV and is a sobering reminder of the need to screen all adults for HIV infection. Additionally, further invasive diagnostic testing may be required to guide therapy in patients with advanced acquired immune deficiency syndrome. This patient's LIP was likely related to long-standing unrecognized HIV disease.

Se presentan ocho casos de neumonia intersticial linfoide (LIP) diagnosticados por nuestro servicio en los ultimos 10 anos. Se analiza la sintomatologia y formas de presentacion de esta enfermedad, los resultados obtenidos con diversas pruebas complementarias, la evolucion de la enfermedad a lo largo del tiempo y la respuesta al tratamiento corticoideo. Por ultimo, se exponen los principales aspectos que se conocen actualmente sobre esta entidad, discutiendo especialmente su etiopatogenia, su posible asociacion a enfermedades autoinmunes y su ocasional degeneracion maligna.

This article continues the discussion of imaging of interstitial lung diseases that was begun in the last issue. We summarize the typical imaging features of idiopathic interstitial pneumonias, emphasizing high-resolution CT. Based on the American Thoracic Society classification system, the idiopathic interstitial pneumonias are discussed under these sections: idiopathic pulmonary fibrosis/usual interstitial pneumonitis, nonspecific interstitial pneumonitis, cryptogenic organizing pneumonia, desquamative interstitial pneumonitis, respiratory bronchiolitis-associated interstitial lung disease, acute interstitial pneumonitis, and lymphoid interstitial pneumonitis.

A 34-year-old woman seronegative for the human immunodeficiency virus presented with recurrent, bilateral pneumothoraces. She also had bibasilar interstitial and alveolar infiltrates, and histologic examination was consistent with lymphocytic interstitial pneumonitis. To our knowledge, this is the first documented case of lymphocytic interstitial pneumonitis presenting with recurrent pneumothoraces.

Polymyositis is associated with a variety of pulmonary manifestations that may complicate an established case, occur simultaneously with the muscle manifestations, or precede the muscle disease. Included are respiratory muscle involvement, aspiration syndromes, and a variety of interstitial lung reactions.

(Chest 1992; 102:263-65) A routine posteroanterior chest radiograph obtamed in a 70-year-old woman showed bibasilar patchy opacities (Fig 1). These infiltrates, which had been noted on a chest radiograph one year earlier, showed slight interval worsening. The patient had never had pulmonary complaints or symptoms. She complained only ofchronic constipation, for which she self-administered mineral oil, and insomnia, for which she took large doses of diazepam each night. The findings from a physical examination, with particular attention to the lungs, were unremarkable. Laboratory data were also unremarkable. Pulmonary function tests were not performed. Computed tomography (CT) of the chest was performed.

The major clinical features of children with acquired immune deficiency syndrome are failure to thrive, opportunistic infections, and lymphoid interstitial pneumonitis. The malignant neoplasm most frequently reported in adults with AIDS is Kaposi sarcoma, but this cancer has been reported in fewer than 5% of children with AIDS. More recently, lymphomas, primarily non-Hodgkin lymphomas, are being reported in increasing numbers in adults infected with human immunodeficiency virus. We describe the clinical, pathologic, and molecular features of an Epstein-Barr virus-associated B cell Burkitt lymphoma in a child with HIV infection.

Pulmonary diseases contribute significantly to the morbidity and mortality in children infected with the human immunodeficiency virus. The wide array of lung diseases spans from early acute bacterial infections, to the lymphoid interstitial pneumonitis/pulmonary lymphoid hyperplasia complex, to opportunistic infections. The unique clinical and histopathologic features of these diseases are reviewed.

• We describe the simultaneous occurrence of Mycobacterium tuberculosis and lymphocytic interstitial pneumonitis in a 54-year-old man. Two drugs were used to treat the tuberculosis, and the patient had clinical improvement but persistent laboratory and chest roentgenographic abnormalities. ( Arch Intern Med 1984;144:1855-1857)

6 children with Acquired Immunodeficiency Syndrome and biopsy proven lymphoid interstitial pneumonitis were treated on a protocol of intravenous gammaglobulin and corticosteroids. Indications for therapy were a clinical history of severe and/or recurrent episodes of respiratory distress responsive to intravenous antibiotics and hypoxemia persisting after resolution of the acute illness. Hypoxemia was defined as a pO2>70 torr on three determinations. Therapy consisted of an initial period of loading with intravenous gammaglobulin. Duration and dosage of loading varied with the severity of the clinical circumstances. Corticosteroids were then initiated at 1-2 mg/kg/day and tapered to 0.75-1.0mg/kg on alternate days within 6-8 weeks. Prior to therapy the mean alveolar-arterial oxygen gradient (AaDO2) was 47 torr and the mean pO2 was 52 torr. After one month of therapy the mean AaDO2 was 21 torr and pO2 was 80 torr. At three months follow-up AaDO2 was 15 torr and pO2 79 torr. In the two patients treated for twelve months, AaDO2 and pO2 remained stable at 16 torr and 89 torr respectively. In vitro immunologic responses were not suppressed by the alternate day corticosteroid treatment.

1791 Lymphoid Interstitial Pneumonitis (LIP) in children with Acquired Immunodeficiency Syndrome (AIDS) prodrome