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Diffuse Alveolar Hemorrhage in a Lupus Patient With Systemic Cryptococcosis
Abstract
Bronchology Student/Resident Case Report PostersSESSION TYPE: Medical Student/Resident Case ReportPRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PMINTRODUCTION: A case of diffuse alveolar hemorrhage (DAH) in a patient with Systemic Lupus Erythematosus (SLE) and Cryptococcus Pneumonia.CASE PRESENTATION: An 18 year old female from Burma with a history of SLE diagnosed 2 years ago, on prednisone, presented with fever and confusion. Lab work revealed methicillin sensitive staphylococcus aureus (MSSA) bacteremia as well as hypocomplementemia [C3- 40 mg/dL (normal 90-180), C4- 8 mg/dL (normal: 8- 36)]. MRI of brain was obtained that showed basal ganglia and periventricular white matter intensities suggestive of lupus vasculitis. Cerebro Spinal Fluid (CSF) analysis was normal. Patient was treated with cefazolin and started on methylprednisolone 1 gm/day for 3 days followed by prednisone taper, on which she showed neurological improvement. However, on 6th hospital day, she developed rapidly progressive respiratory distress with dry cough (no hemoptysis) and hypoxemic respiratory failure requiring 10 L Oxygen via Oxymizer. Chest X-ray & CT-scan chest showed bilateral alveolar infiltrates. Bronchoscopy with Broncho Alveolar Lavage (BAL) revealed hemorrhagic fluid which progressively increased in subsequent aliquots. Culture of the BAL revealed Cryptococcus neoformans which was subsequently also found in blood culture. Patient was also treated with Amphotericin B (0.7 mg/kg per day) plus flucytosine (100 mg/kg per day) for 2 weeks followed by fluconazole for 1 year.DISCUSSION: A large spectrum of pulmonary manifestations can occur in SLE. DAH is an infrequent but potentially serious complication of SLE. Up to a third of patients with diffuse alveolar hemorrhage may not have hemoptysis. We present a rare case of alveolar hemorrhage in a lupus patient who was also found to have systemic cryptococossis. Cryptoccal infections can infect immunocompromised hosts but has not been reported to cause DAH. Patients with severe pulmonary or disseminated cryptococcal disease should be managed as for central nervous system disease, even in the setting of normal findings on spinal fluid examination.CONCLUSIONS: Early BAL is necessary in DAH to rule out infections in addition to establishing the diagnoses.Reference #1: Lara, Abigail R., and Marvin I. Schwarz. "Diffuse alveolar hemorrhage." CHEST Journal 137.5 (2010): 1164-1171.Reference #2: Carmier, D., et al. "Respiratory involvement in systemic lupus erythematosus." Revue des Maladies Respiratoires 27.8 (2010): e66-e78.Reference #3: Kamen, Diane L., and Charlie Strange. "Pulmonary manifestations of systemic lupus erythematosus." Clinics in chest medicine 31.3 (2010): 479.DISCLOSURE: The following authors have nothing to disclose: Abhishek Singla, Pranjal Sharma, Jayanth Adusumalli, Tammy WichmanNo Product/Research Disclosure Information.
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Respiratory involvement in systemic lupus erythematosus (SLE) is not as well-known as the cutaneous, rheumatological and renal manifestations. It occurs frequently but the diagnosis may be difficult because of the heterogeneity of the anatomical and clinical presentations. A precise diagnosis is crucial as new immunosuppressive drugs have considerably improved the prognosis. The pathology involves genetic, endocrine, environmental, pharmacological and immunological factors with a cytotoxic reaction of auto-antibodies against complement, a circulating immune complex reaction and a hyperactivity of B lymphocytes. Respiratory involvement in SLE can be classified in five groups based on the anatomy: pleural involvement, infiltrating pneumonia (lymphoid interstitial pneumonia, bronchiolitis obliterans with organizing pneumonia and acute lupus pneumonitis), airways involvement (upper airways, bronchi), vascular involvement (pulmonary hypertension, acute reversible hypoxaemia, alveolar haemorrhage, and antiphospholipid syndrome), muscular and diaphragmatic involvement (shrinking lung syndrome).Treatment is based, depending upon the type of involvement and its severity, on steroids which may be combined with immunosuppressants and plasmapherisis.
Systemic lupus erythematosus (SLE) is a potentially severe, frequently disabling autoimmune disease with multiorgan involvement and a typically waxing and waning course. SLE is often considered the prototypical autoimmune disease. Although SLE has the potential to affect any organ, the lungs are commonly involved later in the course of the disease in the setting of other organ involvement. Pulmonary disease may complicate SLE and is an important cause of morbidity and mortality. The most common pulmonary manifestation attributable to SLE is pleuritis, but other pleural involvement can be seen, as well as parenchymal disease, pulmonary vascular disease, diaphragmatic dysfunction, and upper airway dysfunction. Finding the true prevalence of lung involvement with SLE is complicated by the high rates of pulmonary infections. This article reviews the diverse clinical symptoms and immunologic pulmonary manifestations of SLE.
Diffuse alveolar hemorrhage (DAH) is often a catastrophic clinical syndrome causing respiratory failure. Recognition of DAH often requires BAL as symptoms are nonspecific, hemoptysis is absent in up to one-third of patients, and radiographic imaging is also nonspecific and similar to other acute alveolar filling processes. Once the diagnosis is established, the underlying cause must be established in order to initiate treatment. This review discusses the diagnosis of the underlying histologies and the clinical entities that are responsible for DAH as well as treatment options.