Acute encephalitis in primary Sjögren’s syndrome: A case report and literature review

ABSTRACT Acute encephalitis is an extremely rare condition in primary Sjogren’s syndrome (pSS), and its characteristics and prognosis remain unclear. Here, we report the case of pSS presented with acute encephalitis. She was admitted to our hospital for acute disturbance of consciousness. Acute encephalitis was diagnosed based on the results of the cerebrospinal fluid test (the increase of leucocyte counts, proteins, and interleukin-6 levels), magnetic resonance imaging, and single-photon emission computed tomography with 99mTc. The infectious aetiologies and underlying malignancies were excluded. Serum anti-Sjogren’s syndrome-related antigen A autoantibody was positive with extremely high titre. The biopsy specimen of her labial salivary gland revealed a focal lymphocytic sialadenitis with a score of grade 4 in the Greenspan grade. She also developed diffuse alveolar haemorrhage during the clinical course. She was diagnosed with pSS complicated with acute encephalitis followed by diffuse alveolar haemorrhage and successfully treated with pulse steroids, high dose of prednisolone and intravenous cyclophosphamide. Our present case and literature review suggest that acute encephalitis associated with pSS can be treatable with the immunosuppressive therapy, and thus early recognition and treatment initiation are important for this life-threatening condition. Thus, pSS should be included in the differential diagnosis of unexplained encephalitis. Notably, our case characteristically showed diffuse alveolar haemorrhage, adding new insights into the pathogenesis of acute encephalitis associated with pSS that capillaritis might be the underlying cause of this condition.

Pathologically confirmed diffuse alveolar haemorrhage in lymphangioleiomyomatosis

A 40-year-old woman was referred to pulmonology after presenting with dyspnoea and self-limiting haemoptysis. Chest CT revealed diffuse ground glass opacities and small thin-walled cysts. Bronchoalveolar lavage cultures were negative and cytology revealed haemosiderin-laden macrophages. Transthoracic echocardiogram was normal. Connective tissue disease and vasculitis work-up were negative. Vascular endothelial growth factor-D level was indeterminate. Lung function was normal. She underwent video-assisted thoracoscopic lung biopsy. In addition to findings consistent with lymphangioleiomyomatosis, histopathological examination identified haemosiderosis without capillaritis, confirming a diagnosis of diffuse alveolar haemorrhage in the context of the associated clinical and radiographic features. Follow-up imaging after 5 months showed resolution of the diffuse ground glass opacities. Pharmacotherapy with sirolimus was not initiated due to absence of deterioration in pulmonary function. Diffuse alveolar haemorrhage in patients with lymphangioleiomyomatosis is a rare but important presentation. The few previously reported cases progressed to respiratory failure requiring mechanical ventilation.

Diffuse alveolar haemorrhage: a rare complication of clopidogrel use

Diffuse alveolar haemorrhage (DAH) has been reported as a rare complication of clopidogrel use and is usually a diagnosis of exclusion. We describe the case of an 88-year-old Native American woman who presented with acute hypoxic respiratory failure with CT scan of the chest showing diffuse bilateral ground-glass opacities. She had been on clopidogrel for 6 months for a carotid artery stent. Bronchoscopy with bronchoalveolar lavage and transbronchial biopsies revealed DAH. Infectious and autoimmune work-up were all negative. Clopidogrel was stopped and high-dose steroids were started. Her symptoms gradually improved until she was discharged from the hospital. The differential DAH is broad. Anticoagulant-induced DAH should be part of the differential diagnosis, and is usually a diagnosis of exclusion.

Diffuse alveolar haemorrhage and myocardial infarction: life-threatening effects from self-injected hyaluronic acid dermal filler

Hyaluronic acid (HA) is a widely used dermal filler for soft tissue augmentation. We described a case of a 38-year-old transwoman who presented with sudden onset of severe respiratory distress following self-injection of HA dermal filler. She developed multiple episodes of pulmonary haemorrhage, and her chest X-ray showed diffuse ground-glass opacities consistent with diffuse alveolar haemorrhage (DAH). There were no relevant drugs or past medical histories. Anti-nuclear antibodies and rheumatoid factor were negative. Initially, the pulmonary haemorrhage episodes and ventilation requirement improved with systemic steroid, however she subsequently developed acute myocardial infarction with progressive clinical deterioration leading to death. To the best of our knowledge, this is the first HA-related DAH with myocardial infarction reported with a fatal outcome. This case highlights the importance of awareness and the necessity of having a high suspicion of DAH in patients with history of illicit HA dermal filler use.

Diffuse alveolar haemorrhage secondary to haemophilus influenzae in a vaping patient

Diffuse alveolar haemorrhage (DAH) is known to occur from several infectious and non-infectious aetiologies. Among the infectious aetiologies, Haemophilus influenzae, an organism known to cause community-acquired pneumonia, has not been reported in association with DAH prior to this case. On the other hand, vaping, an evolving epidemic concern, has been linked to various types of lung injury, including DAH. However, DAH related to vaping is only limited to several case reports. Our case describes H. influenzae pneumonia with DAH in a patient known to have vaped until the night prior to elective lobectomy for lung cancer and developed DAH within 24 hours of hospitalisation. He subsequently recovered with treatment. DAH requires immediate recognition, and healthcare providers need to be aware that neither haemoptysis nor decrease in haemoglobin level is necessarily associated. Prior to diagnosis, empirical treatment with intravenous steroids and antibiotics can be life-saving.

Fulminant granulomatosis with polyangiitis presenting with diffuse alveolar haemorrhage following COVID-19

A 40-year-old man developed granulomatosis with polyangiitis (GPA) following a mild case of COVID-19. Initially, he experienced mild migrating joint pain for 2 months prior to testing positive for SARS-CoV-2 but dramatically worsened following resolution of his infection. The pain continued to progress until he suddenly develope haemoptysis, prompting him to present to a local hospital. The diagnosis of diffuse alveolar haemorrhage secondary to GPA was confirmed with labs, imaging and histopathology. Precipitous deterioration of GPA with concurrent COVID-19 infection indicates a possible temporal relationship. Since the onset of the pandemic, SARS-CoV-2 has been anecdotally associated with the development of various connective tissue disorders. The overlapping clinical presentations and similar appearance on lung imaging present clinicians with a diagnostic challenge. This underscores the importance of having a high index of suspicion of autoimmune diagnoses in patients who present with new or worsening findings following a COVID-19 infection.

Diffuse alveolar haemorrhage in the setting of eosinophilic pneumonia

A 65-year-old woman who presented with a constellation of symptoms, including cough with haemoptysis, fever, chills and hypoxia along with weight loss, was found to have diffuse alveolar haemorrhage. After a myriad of investigations returned normal, an open lung biopsy was performed, which revealed the diagnosis to be subacute eosinophilic pneumonia. This is one of its kind of rare presentations where eosinophilic pneumonia presents as diffuse alveolar haemorrhage and has been reported only five times prior to this.