Isolated acute lupus pneumonitis as the initial presentation of systemic lupus erythematosus in an 8-year-old girl
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Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which has broad pleuropulmonary manifestations. One of the rare and mortal complications is acute lupus pneumonitis, which is reported very rarely, especially in childhood. Herein, we report an 8-year-old girl with isolated acute lupus pneumonitis as the initial presentation that required a lung biopsy for diagnosis. Although she had improvement with the administration of steroids, steroid treatment was reduced due to the drug’s side effects resulting in the addition of azathioprine and mycophenolate mofetil to the treatment regimen. After the new regimen failed to result in clinical improvement, hydroxychloroquine treatment was started and a significant improvement was observed. Acute lupus pneumonitis is an uncommon manifestation of SLE and diagnosis may be difficult in patients without other organ involvement.
KeywordsAcute lupus pneumonitis Child Hydroxychloroquine Lung biopsy
Systemic lupus erythematosus (SLE) is a multisystem autoimmune connective tissue disease and commonly presents with arthritis, serositis, cutaneous manifestations, glomerulonephritis, and hematological and central nervous system involvement [1, 2]. Pulmonary involvement in SLE includes pulmonary hemorrhage, pulmonary hypertension, acute lupus pneumonitis, chronic interstitial pneumonitis, shrinking lung syndrome, pulmonary vasculitis, pulmonary embolism, bronchiolitis obliterans, cavitating pulmonary nodules, opportunistic pulmonary infections, and pleuritis, the most common [1, 3, 4]. Childhood onset SLE constitutes 20% of all lupus patients and pulmonary involvement has been reported in up to 80% of cases [5, 6, 7, 8]. Acute lupus pneumonitis is rare in SLE and can be very difficult to diagnose in patients without any previous diagnosis of SLE. Herein, we report an 8-year-old girl with isolated acute lupus pneumonitis as the initial presentation.
Acute lupus pneumonitis is a rare manifestation of SLE which is reported in 1–4% of SLE patients with a high mortality rate [4, 10]. Therefore, early diagnosis and treatment are essential in these patients. Our patient had many difficulties for an accurate diagnosis. She had only pulmonary involvement without any other systemic symptoms, resulting in an initial diagnosis of SLE. In this case, the major concern was preventing pulmonary infections. Infections are the main cause of death in SLE patients . All microbiological cultures were negative including tuberculosis. The incidence of pulmonary tuberculosis is higher in SLE patients with a 6–15-fold higher risk than in the normal population [12, 13, 14]. BAL cultures were sterile and pulmonary infections were excluded. Viral pneumonias such as CMV were also excluded. Pulmonary hemorrhage was the other differential diagnose but was eventually excluded as she had no hemoptysis, anemia and was without hemosiderin-laden macrophages in BAL. Systemic vasculitis was another possibility but was excluded as there was no vasculitic rash, glomerulonephritis, or positive antibodies supporting vasculitis. Lung biopsy was required to fulfill the SLE criteria and is an invasive procedure in childhood.
High-dose steroids, azathioprine, cyclophosphamide, mycophenolate mofetil, and other immunosuppressive agents can be used in the treatment of acute lupus pneumonitis [4, 15, 16]. There are no clinical trials for this treatment and current treatment regimens are based solely on case reports and small investigative series . Pulmonary function tests and radiological findings of our patient were improved and immunological markers became negative following steroid treatment. Due to the side effects of steroids, a reduced dosage was necessary, resulting in a deterioration of pulmonary functions and radiological findings concurrent with positive immunological markers. This was another finding that confirmed our diagnosis. Azathioprine and mycophenolate mofetil were added, but a significant improvement was observed only after the addition of hydroxychloroquine treatment. Hydroxychloroquine treatment is used for long-term protective effects against SLE-related organ damage; however, the percentage of SLE patients receiving hydroxychloroquine treatment was reported to be very low [16, 17]. Although hydroxychloroquine is not mentioned as a first-line therapy option, it can be use safely in acute lupus pneumonitis in children. We did not observe any side effects due to hydroxychloroquine treatment in this patient. Another explanation may be for failure of azathioprine and mycophenolate mofetil and success of hydroxychloroquine as hydroxychloroquine is a weak immunomodulator compared to other two agents or it was just delayed effect of azathioprine and mycophenolate mofetil which lead to success.
In the English literature, patients presenting with acute lupus pneumonitis as the initial presentation have other manifestations of SLE such as photosensitivity, alopecia, oral ulcers, malar rash, and glomerulonephritis [2, 4, 13]. Our patient had only pulmonary manifestation which made the diagnostic difficult and had to be diagnosed in accordance with other organ involvement.
In conclusion, interstitial lung involvement as the sole symptom in SLE is very rare, especially in childhood. Acute lupus pneumonitis is a rare and mortal manifestation of SLE that sometimes necessitates invasive procedures such as lung biopsy for a diagnosis. Hydroxychloroquine treatment can be used in children with acute lupus pneumonitis when steroid treatment is contraindicated due to side effects.
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Conflict of interest
The authors TSE, ATA, YO, DGY, NB, and OB declare that they have no conflict of interest.
Research involving human participants and/or animals
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
Informed consent was obtained from the parents.
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