INTRODUCTION: Acute Lupus Pneumonitis is an uncommon (1-12 %) manifestation of Systemic Lupus Erythematosus (SLE)1. It is usually characterized by fever, cough, pleuritis, dyspnea, pulmonary infiltrates, hypoxia, and basilar rales on a background of no apparent infection1. A high index of suspicion is needed at presentation as patients can rapidly deteriorate resulting in increased morbidity and mortality. We report a case of a 22 year old female with acute fulminant lupus pneumonitis who had no known history of SLE.
CASE PRESENTATION: A 22 year old AAF with past medical history of seizure disorder diagnosed a few months prior to admission presented to the emergency department (ED) with complaints of generalized weakness and anorexia for three weeks. On further questioning she also complained of cough and mild shortness of breath. Her initial vital signs were: heart rate 120 bpm, blood pressure 88/50 mm/Hg, respiratory rate 20/min, she was afebrile and oxygen saturation was 92% on room air. Physical examination revealed a thin chronically ill lady in mild respiratory distress with bilateral coarse crackles. Rest of examination was unremarkable. Her initial laboratory tests showed WBC 4.8 K/mm3, Hct 29 %, Platelets 148,000/L, sodium 127 mmol/L, chloride 111 mmol/L, creatinine 1.3 mg/dl, INR 1.56, PTT 50sec, Fibrinogen 127 mg/dl, Lactate 3.2 mmol/L, AST 137 U/L, LDH 1306 U/L and CPK 1886 U/L. Chest X-ray showed bilateral pulmonary infiltrates and small pleural effusions. Patient received intravenous fluids for hypotension. Her shortness of breath started getting worse. A CT scan of chest was done which showed diffuse bilateral infiltrates. Her respiratory status progressively worsened requiring intubation. Repeat chest X-ray showed worsening bilateral infiltrates. The next day she went into circulatory shock requiring vasopressors and developed multiorgan failure with DIC. Her repeat lab results showed PTT of 250 sec, INR 10, platelets count of 100,000/L and fibrinogen of 50 mg/dl. An echocardiogram showed moderate pericardial effusion with probable hemodynamic compromise. Unfortunately, the patient went into cardiac arrest just after the echo was completed. ACLS was performed. Pericardiocentesis was also attempted but the patient expired. All cultures including blood, deep tracheal aspirate, urine and fungal cultures remain negative. Urine histoplasma antigen, serology for HIV and hepatitis, urine Legionella antigen, and silver stain for P. jeroveci in tracheal aspirate were also negative. Rheumatology panel sent from ED on her admission day came back later showing: Anti RNP: 413(0-99), Anti sm: 505(0-99), Aldolase: 68(1.2-7.6), Anti Smith: 536(0-99).
DISCUSSION: Patients with SLE can present with a variety of lung manifestations which include pleuritis, acute pneumonitis, pulmonary hypertension, shrinking lung syndrome and pulmonary hemorrhage(1). Acute Lupus Pneumonitis (ALP) is one of such presentations with very high morbidity and mortality rate (nearly 50%)(1). Usually it is a diagnosis of exclusion when a complete work up fails to isolate an infectious pathogen or alternative diagnosis. Pathologic examination in ALP reveals acute alveolar wall injury, alveolar edema, hyaline membrane formation and immunoglobulin and complement deposition(2). Late inspiratory crackles, CT scan, and BAL help in diagnosis of alveolitis. ALP needs prompt intervention as early therapy with steroids and immunosuppressants have been shown to improve outcomes(1).
CONCLUSIONS: We suggest clinicians to consider lupus as a differential in afebrile young patients presenting with diffuse lung disease without any obvious etiology.
Reference #1 Matthay, RA, Schwarz, MI, Petty, TL, et al. Pulmonary manifestations of systemic lupus erythematosus: review of twelve cases of acute lupus pneumonitis. Medicine ( Baltimore) 1975; 54: 397
Reference #2 Lawrence, EC. Systemic lupus erythematosus and the lung. In: Systemic Lupus Erythematosus, Lahita, EG (Ed), John Wiley and Sons, New York 1987.
DISCLOSURE: The following authors have nothing to disclose: Dipen Kadaria, Nicole Pant, Waqas Chishti, Jose Yataco
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