Systemic lupus erythematosus presenting as acute lupus pneumonitis in a young female

Abstract

Acute lupus pneumonitis is a rare initial presentation of systemic lupus erythematosus (SLE). We report a 19-year-old female presenting with fever and recurrent hemoptysis with radiological evidence of parenchymal lung involvement with mild pleural effusion. Subsequent development of malar and discoid rash with anti-nuclear antibodies (ANA) and anti-dsDNA positivity clinched the diagnosis. Her clinical signs and symptoms resolved with a course of intravenous pulse methyl-prednisolone along with radiological resolution.

Introduction

Acute lupus pneumonitis (ALP) is a rare initial presentation of systemic lupus erythematosus (SLE). We report a 19-year-old female presenting with fever and recurrent hemoptysis with radiological evidence of parenchymal lung involvement with mild pleural effusion.

Case Report

A 19-year-old previously healthy girl presented with high grade fever with chills and recurrent episodes of hemoptysis for the past one month along with mild dyspnea, dry cough, pleuritic chest pain and fatigue. On physical examination, she was febrile with tachycardia, and coarse crepitations in right upper lung zone. Day 1 blood reports revealed anemia (hemoglobin 9 gm/dL, normocytic normochromic), leukopenia (total leukocyte count 2700/mm³), thrombocytopenia (platelet count 1 × 105/mm³) and raised erythrocyte sedimentation rate (ESR) with normal C-reactive protein (CRP). Serum biochemistry, urine analysis, urine culture were unremarkable. Chest radiograph showed opacity in the right upper lobe with air bronchograms. Ultrasonography of the abdomen was normal. Sputum culture, blood culture, sputum microscopy for acid fast bacilli, Mantoux test and HIV serology were negative. The patient was started on intravenous antibiotics and supportive care. However, when fever persisted beyond 96 hours, a high-resolution computed tomography (HRCT) scan of thorax was done. It showed consolidation of the right upper and lower lobes, left lower lobe, patchy infiltrative changes in both lungs, a mild right-sided pleural effusion with few small mediastinal lymph-nodes in pre-tracheal and right tracheo-bronchial locations [Figure 1]. A diagnostic thoracocentesis revealed an exudative hemorrhagic fluid with normal pleural fluid adenosine deaminase levels. The pleural fluid did not grow any bacteria and was negative for malignant cells. Fiber-optic bronchoscopy with bronchoalveolar lavage (BAL) fluid analysis showed only pus cells and epithelial cells. Bone marrow examination was unremarkable. On Day 6, the patient developed a fixed flat rash over the malar prominences and bridge of the nose sparing the naso-labial folds and discoid rash on the back [Figure 2] with continuing fever. Anti-nuclear antibody (ANA) testing was done and found to be positive (titer 1:320, homogeneous pattern), anti-ds-DNA was weakly positive, while perinuclear and cytoplasmic anti-neutrophil cytoplasmic antibody (p-ANCA and c-ANCA, respectively) were negative. Serum C3 and C4 levels were decreased (40 mg/dl and 8 mg/dl, respectively). Thus, a diagnosis of SLE presenting with acute pneumonitis was made.

Figure 1.

(a and b) HRCT thorax showing consolidation and patchy infiltrative changes in both lungs (Rt. > Lt.) along with air bronchograms

Figure 2.

(a) Fixed flat rash over the malar prominences and the bridge of the nose sparing the naso-labial folds. (b) Erythematous patches with scaling on back

On Day 7, the patient was started on 1 gram intravenous methyl prednisolone once a day for 3 days, followed by tablet hydroxycholoquine 400 mg daily and tablet prednisolone 1 mg/kg daily for 6 weeks with gradual tapering of prednisolone to a maintenance dose of 10 mg daily. There was dramatic clinical improvement and findings on chest radiography completely resolved on Day 15. She is currently being followed up on an outpatient basis with monitoring of her hematologic and renal profile along with urine analysis at regular intervals.

Discussion

Though pleuritis is common in SLE (50-83% on autopsy), severe parenchymal involvement is less frequently seen.[1] ALP occurs in 1-4% patients with SLE and is one of the most dreaded life-threatening syndromes complicating SLE, with short-term mortality of 50-90%.[1,2,3] Acute injury to alveolar capillary unit may be the central pathology in ALP. Clinical presentation may be non-specific and may include cough with or without expectoration, sometimes hemoptysis, pleuritic pain, dyspnea, hypoxemia, fever, chills along with constitutional symptoms of malaise, fatigue, anorexia. Lung involvement may be unilateral or bilateral. Physical exam may reveal crepitations, bronchial breath sounds or decreased breath sounds in the basal regions owing to effusion. As in this case, diagnosis may be difficult in an undiagnosed patient of SLE where other organ involvement, antibodies or signs and symptoms are either absent or still evolving. Chest radiograph may reveal patchy infiltrates, airspace opacities with or without pleural effusion while ground glass appearance or a honeycomb appearance maybe appreciable in HRCT.[4] It is logical to think of an infectious etiology first and do the relevant work up; if that comes back negative a pneumonitis should be suspected. This is extremely important because the two entities have a similar presentation but very different treatments. Also, it is critically important to differentiate ALP from diffuse alveolar hemorrhage (DAH) which may have a very similar presentation and radiographic findings with an almost equally grave prognosis. Both ANA and anti-dsDNA testings are commonly positive in ALP. ESR, CRP or high sensitivity-CRP (hs-CRP) may be used to support clinical suspicion. Unlike ESR, rise of CRP (or hs-CRP) is only modest, at best in active SLE without infection while a high hs-CRP level (>5-6 mg/dL) is a strong predictor of infection. Also, ESR/CRP ratios >15 suggest lupus flare, while ratios <2 suggest infection.[5] A lung biopsy is helpful to differentiate between ALP and DAH, but is not recommended in these patients.

Declaration of Patient Consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.