A Collection of Conditions Affecting the Lungs

Fungal Pneumonia Caused by Candida dubliniensis

Rafael Ilyayev, MD
Refua Shlema Medical, Brooklyn, New York

Mohamed Ali Mohamed, MD
Wayne State University, Detroit, Michigan

Hassan Farhat, MD
New York Community Hospital, Brooklyn, New York

A 45-year-old man was admitted to the hospital with a weeklong history of fever, chills, worsening dyspnea on exertion, increasing generalized weakness, and a decrease in functional capacity. He had a past history of HIV/AIDS and was on antiretroviral therapy, with a CD4 cell count of 25/mm³ (reference range, >500/mm³) on admission. He was febrile (39.3°C), with a heart rate of 108 beats/min, a respiratory rate of 22 breaths/min, blood pressure of 110/70 mm Hg, and oxygen saturation of 92% on room air. 

Two months prior to admission, the patient had been admitted and treated for Pneumocystis jirovecii (previously known as Pneumocystis carinii) pneumonia (PCP) that had been complicated with the development of bilateral pneumothorax.

Diagnostic tests. Posteroanterior chest radiography findings showed multiple bilateral pulmonary nodules measuring as much as 2 cm in diameter without evidence of cavitation, but with a solitary cavitary lesion in the left upper lobe (Figure 1).

Laboratory test results were significant for leukocytosis (white blood cell count, 14,900/µL; 87.7% neutrophils, 6% lymphocytes, and 0.2% eosinophils). His fasting blood glucose level was 135 mg/dL (reference range, 70-110 mg/dL). His creatinine level was 1.9 mg/dL (reference range, 0.6-1.2 mg/dL) and his blood urea nitrogen level was 19 mg/dL (reference range, 8-23 mg/dL).

Results of liver function tests were as follows: total bilirubin, 0.2 mg/dL (reference range, 0.3-1.2 mg/dL); aspartate aminotransferase, 16 U/L (reference range, 10-30 U/L); alanine aminotransferase, 323 U/L (reference range, 10-40 U/L); and alkaline phosphatase, 347 U/L (reference range, 30-120 U/L).

The patient was started empirically on an intravenous broad-spectrum antibiotic regimen of vancomycin and doripenem. Atovaquone was added to cover for PCP but with no significant improvement. Computed tomography (CT) scan of the chest (Figures 2 and 3) showed multiple new bilateral pulmonary nodules, along with increased multifocal small patchy infiltrates and new mediastinal lymphadenopathy. He remained febrile despite the trial of antibiotics and PCP treatment.

Given the lack of improvement of the patient’s condition despite broad-spectrum antibiotics, and given the development of multiple new pulmonary nodules, bronchoscopy with bronchoalveolar lavage (BAL) was performed. The results of BAL specimen examination were negative for acid-fast bacilli (AFB), P jirovecii, malignant cells, and pyogenic organisms but showed moderate growth of Candida dubliniensis. Sputum examination also showed profuse growth of C dubliniensis, confirming the diagnosis of fungal pneumonia caused by the organism.

A cardiothoracic surgeon was consulted to assess the patient for a transthoracic biopsy, but the procedure was withheld because of the patient’s very high surgical risk given his history of bilateral pneumothorax. Treatment with 100 mg/d of intravenous micafungin was initiated, leading to significant clinical improvement, including resolution of the fever and improvement of the cough. The patient was discharged from the hospital on oral voriconazole and was instructed to follow up with the clinic for outpatient interval chest CT scans to ensure resolution of the lung densities.

Outcome of the case. A follow-up chest CT scan at 3 months showed the lymphadenopathy to be unchanged, but the multiple bilateral pulmonary nodules appeared to have minimally decreased in size since the previous CT scan. At 6 months, a chest CT scan showed partial resolution of the multiple bilateral irregular nodules (Figures 4 and 5). A chest CT scan at 12 months showed interval resolution of most of the pulmonary nodules and resolution of the mediastinal adenopathy (Figures 6 and 7).

Discussion. Some Candida species (such as Candida albicans) are commensal organisms in healthy individuals and are part of the normal flora of the oral cavity, the gastrointestinal tract, the vaginal tract, and the surface of the skin. However, C dubliniensis is a pathogenic organism that is rarely found in healthy individuals; in immunocompromised persons, it can cause local infection and in rare cases fulminant pneumonia or disseminated candidemia. In most reported cases of infection, C dubliniensis has been susceptible to antifungal medications.¹

In recent years, C dubliniensis has been isolated more often in the respiratory tract of immunocompromised patients. Most fungal pneumonia cases in immunocompromised patients are due to infection with P jirovecii, Aspergillus species (especially Aspergillus fumigatus), and Cryptococcus neoformans. Fungal pneumonia due to C dubliniensis is a rare entity, with very few cases having been reported.

The diagnosis of fungal pneumonia is challenging given that the infection is very rare and given the difficulty of establishing whether the presence of Candida species in isolates represents colonization or infection. However, in an immunocompromised patient presenting with pneumonia, a vigilant search for unusual organisms should be considered, particularly in a patient whose infection is not responding to broad-spectrum antibiotics. The use of bronchoscopy and high-resolution CT scans can facilitate this process and increase the likelihood of identifying a patient with fungal pneumonia.²

Radiographically, C dubliniensis pneumonia can present with a miliary pattern along with localized or diffuse bronchopneumonia. However, a nodular pattern and the presence of cavitary masses also have been reported.³

Fungal pneumonia with C dubliniensis should be included in the differential diagnosis of multiple pulmonary nodules in an immunocompromised patient.

REFERENCES:

1. Sullivan DJ, Moran GP, Pinjon E, et al. Comparison of the epidemiology, drug resistance mechanisms, and virulence of Candida dubliniensis and Candida albicans. FEMS Yeast Res. 2004;4(4-5):369-376.
2. von Eiff M, Roos N, Fegeler W, et al. Hospital-acquired candida and aspergillus pneumonia—diagnostic approaches and clinical findings. J Hosp Infect. 1996;32(1):17-28.
3. Kalkanci A, Kokturk N, Senol E, et al. Could Candida dubliniensis be involved in lung fungus balls? Rev Iberoam Micol. 2005;22(3):157-159.

NEXT: Legionnaire Disease

Legionnaire Disease

Carlos Ruz-Pau, MD, and Jorge Parellada, MD
Orlando Regional Medical Center, Orlando, Florida

Zoran Pavlovic, MS-IV
University of Central Florida, Orlando, Florida

A 56-year-old woman with a history of type 2 diabetes mellitus, hypertension, and hyperlipidemia presented to the emergency department with abdominal pain. She reported constant left-sided abdominal pain that had progressively worsened for a week, with associated profuse nonbloody diarrhea.

History. She reported having had mild shortness of breath with prolonged exertion over the past 6 weeks, but it had not been significant enough to concern her. She denied a history of HIV or having HIV risk factors. She denied cough, fevers, chills, sick contacts, and recent domestic or international travel but reported having noted a foul odor in her apartment that had intensified since the beginning of the summer months.

Physical examination. On initial evaluation, the patient was noted to be hypoxic, requiring high levels of oxygen via nasal cannula in order to maintain oxygen saturation above 90% as measured by pulse oximetry (Spo₂). She was afebrile, with a blood pressure of 94/53 mm Hg, a pulse rate of 90 beats/min, a respiratory rate of 32 breaths/min, and an Spo₂ of 90% on 8 L/min of oxygen via nasal cannula.

She was noted to have increased respiratory effort with decreased breath sounds in all right lung fields, left basilar crackles, and coarse breath sounds on the left middle and left upper lung fields. Cardiac examination findings were normal. Her abdomen was tender to light palpation in left upper and left lower quadrants without rebound tenderness or guarding. No deficits were appreciated on neurologic examination except for marked delay in responses to simple questions.

Diagnostic tests. Laboratory studies revealed the following values: sodium, 138 mEq/L (reference range, 136-142 mEq/L); potassium, 3.1 mEq/L (reference range, 3.5-5.0 mEq/L); chloride, 100 mEq/L (reference range, 96-106 mEq/L); bicarbonate, 24 mEq/L (reference range, 21-28 mEq/L); urea nitrogen, 51 mg/dL (reference range, 8-23 mg/dL); lactate, 10.8 mg/dL (reference range, 5.0-15.0 mg/dL); and creatinine, 3.04 mg/dL (reference range, 0.6-1.2 mg/dL).

Arterial blood gas tests revealed the following values: pH, 7.48 (reference range, 7.35-7.45); partial pressure of carbon dioxide, 35 mm Hg (reference range, 35-45 mm Hg); partial pressure of oxygen, 51 mm Hg (reference range, 80-100 mm Hg); and bicarbonate, 26.1 mEq/L (reference range, 21-28 mEq/L). The results of urinalysis and urine drug screening tests were both negative.

A chest radiograph (Figure 1) showed asymmetric patchy alveolar interstitial infiltrates and middle to lower lobe consolidation, findings that were visualized on a chest computed tomography (CT) scan (Figure 2).

The patient was tested for the Legionella pneumophila antigen, the results of which were positive, leading us to the diagnosis and proper management.

Discussion. The United States has an annual incidence of 8000 to 18,000 new cases of Legionnaire disease. Most cases occur during the late spring or early fall, and there is a mildly increased incidence in northern states.^1,2 The responsible pathogen was identified in 1976 as L pneumophila, although this bacterium is one of 42 species constituting 64 serogroups.^2,3 It is a small, gram-negative, catalase-positive, weakly oxidase-positive, aerobic, waterborne bacterium that is nonmotile and often lives in symbiosis with various amoebic species that are common residents of water-rich environments. These water-rich environments include the cooling systems found in hotels, apartments, cruise ships, and hospitals, as well as showers, decorative fountains, humidifiers, and whirlpool spas, among other structures, in which the bacteria can survive at an optimal temperature.

Humans often become infected through inhalation of aerosolized particles or via aspiration of contaminated liquid. Legionella infection typically is not transferrable directly from human to human.⁴ Our patient became infected via her window air conditioner that she had begun to use during the late spring.

Once infection has occurred, Legionella, which is an obligate intracellular organism, is ingested by macrophages, where the bacteria disrupt the endosomal-lysosomal pathway and replicate inside of phagosomes instead of being destroyed. The macrophage then dies, and the bacteria escape and go on to infect other macrophages.⁵

Legionella pneumonia has a 2- to 14-day incubation period, and the first symptoms to appear are usually a nonproductive cough, high fever, and myalgias, with approximately a third of patients also developing dyspnea and pleuritic chest pain. Gastrointestinal tract symptoms occur in up to half of patients and consist of abdominal pain, nausea, vomiting, and nonbloody diarrhea. In some severe cases, patients may have altered mental status and ensuing respiratory failure, which is often the cause of increased mortality.⁶

Our patient failed to show any of the typical symptoms of cough, high fever, myalgias, or chest pain and instead presented with gastrointestinal tract symptoms that could have easily misdirected her initial management, thus highlighting the importance of taking a detailed history and performing a thorough physical examination. This patient began using an old window unit air conditioner; she had noticed a foul odor in her apartment that she later found to be coming from aerosolized water particles being dispersed from the air conditioner.

Legionnaire disease can have many nonspecific findings such as elevated levels of liver enzymes, erythrocyte sedimentation rate, C-reactive protein, and ferritin, as well as some mildly more-specific findings such as hyponatremia secondary to syndrome of inappropriate antidiuretic hormone secretion.

Imaging should not be relied on solely for diagnosis but can be useful for confirmation. Chest radiographs will typically show rapidly progressive asymmetric patchy alveolar interstitial infiltrates and middle to lower lobe consolidation, which can be better visualized with a CT scan,⁷ as with our patient. The definitive diagnostic method for Legionnaire disease is via culture of respiratory secretions, whether sputum or pleural fluid. However, culture results may take 3 to 5 days and require a specialized chocolate yeast extract medium with cysteine and iron for growth.⁶ In many hospitals, the Legionella urine antigen test is used due to its rapid availability, low cost, and specificity of 99%. However, the urine antigen test is only for L pneumophila serogroup 1, which is responsible for most cases, but it does not detect disease caused by other serogroups.⁶

Once the diagnosis has been made, patients are often treated with macrolide, tetracycline, or fluoroquinolone antibiotics. The goal is to choose an antibiotic with adequate intracellular penetration due to the intracellular nature of Legionella. The most commonly used antibiotics are azithromycin, doxycycline, and levofloxacin. Typically, antibiotic therapy is started in the hospital intravenously, with the therapy being switched to oral antibiotics once a patient’s condition has stabilized. The duration of therapy is usually 3 to 5 days for azithromycin and 5 to 10 days for levofloxacin, unless there is severe disease with sepsis or if the patient is immunocompromised, in which treatment is extended.⁶ Prognosis is highly variable and often depends on a patient’s comorbid conditions as well how quickly treatment is initiated, with increasing mortality in nosocomial infections.⁶ Therefore, prevention is of the utmost importance and can be done by superheating water sources to 70°C to 80°C or utilizing copper-silver ionization units, or UV light, all of which eradicate the Legionella bacteria.⁸ 

References:

1. Marston BJ, Plouffe JF, File TM Jr, et al. Incidence of community-acquired pneumonia requiring hospitalization: results of a population-based active surveillance study in Ohio. Arch Intern Med. 1997;157(15):1709-1718.
2. Brunkard JM, Ailes E, Roberts VA, et al. Surveillance for waterborne disease outbreaks associated with drinking water—United States, 2000–2008. MMWR Surveill Summ. 2011;60(12):38-68.
3. Kozak-Muiznieks NA, Lucas CE, Brown E. Prevalence of sequence types among clinical and environmental isolates of Legionella pneumophila serogroup 1 in the United States from 1982 to 2012. J Clin Microbiol. 2014;​52(1):201-211.
4. Woo AH, Goetz A, Yu VL. Transmission of Legionella by respiratory equipment and aerosol generating devices. Chest. 1992;102(5):1586-1590.
5. Newton HJ, Ang DKY, van Driel IR, Hartland EL. Molecular pathogenesis of infections caused by Legionella pneumophila. Clin Microbiol Rev. 2010;​23(2):​274-298.
6. Cunha BA, Burillo A, Bouza E. Legionnaires’ disease. Lancet. 2016;​387(10016):​376-385.
7. Tan MJ, Tan JS, Hamor RH, File TM Jr, Breiman RF. The radiologic manifestations of Legionnaire’s disease. Chest. 2000;117(2):398-403.
8. Borella P, Bargellini A, Marchegiano P, Vecchi E, Marchesi I. Hospital-acquired Legionella infections: an update on the procedures for controlling environmental contamination. Ann Ig. 2016;28(2):98-108.

NEXT: Rheumatoid Lung Nodules

Rheumatoid Lung Nodules

Juan Jaller-Char, MD, and Joan Morales Lappot, MD
University of Central Florida College of Medicine, Orlando, Florida

Hammad Bhatti, MD, and Sayed K. Ali, MD
Orlando Veterans Affairs Medical Center, Orlando, Florida

A 67-year-old man with a long history of tobacco use and rheumatoid arthritis (RA) presented to our clinic for a follow-up appointment. He had received a diagnosis of RA approximately 15 years ago and currently was on a regimen of a low-dose corticosteroid with methotrexate 3 times a week. He had recently visited an urgent care clinic for a cough, where a chest radiograph had revealed multiple lung nodules. He was asked to follow up with his primary physician.

History. The man denied any alcohol or illicit drug use. He worked in a grocery store stocking vegetables. He was married and lived with his wife and 2 dogs. His mother had died after a cardiac event, and his father had died as a result of a stroke. He denied a family history of lung cancer or other cancers.

Physical examination. On presentation, he was afebrile with stable vital signs. Cardiac examination findings were normal, and his lungs were clear to auscultation. He had multiple joint deformities as a result of RA, but the rest of the physical examination findings were unremarkable. No subcutaneous nodules were appreciated on examination of the skin.

Diagnostic tests. A chest radiograph in our clinic verified the presence of pulmonary nodules (Figure 1). A subsequent computed tomography (CT) scan of the chest also revealed multiple cavitating nodules, the largest measuring 1.5 cm in diameter (Figure 2). These nodules had not changed in size from a previous chest CT scan performed approximately 1 year ago. Results of a bronchoscopy with bronchoalveolar lavage were negative for acid-fast bacilli, fungi, and malignant cells. A positron-emission tomography (PET) CT was performed but failed to show uptake. Biopsy results of the largest nodule were negative for malignant cells.

The patient received a diagnosis of rheumatoid lung nodules.

Discussion. Lung nodules are a rare but specific finding of RA. They occur more frequently in men who are positive for rheumatoid factor, in smokers, and those with subcutaneous nodules.¹ They can present prior to or after the manifestations of RA. Rheumatoid lung nodules are generally located in subpleural areas or in association with interlobular septa. They usually range in size from 0.5 mm to 7 cm.¹ They can be single or multiple and sometimes can resolve spontaneously. They are not directly related to the course of RA.¹

It is essential to exclude malignancy and infection in patients presenting with pulmonary lung nodules, especially in patients with risk factors such as smoking or who present with a single nodule. Follow-up imaging is often necessary, and sometimes needle aspiration may be required to exclude malignancy.

Rheumatoid lung nodules are often asymptomatic and rarely cause complications. PET CT, although not very accurate, can sometimes be used to detect a potential malignancy in such inflammatory conditions.²

Outcome of the case. Given the stable nature of the nodule over the course of 2 years, the patient was offered reassurance and education about his condition. A follow-up appointment was arranged for 6 months later. The current RA treatment appeared to be controlling his symptoms and therefore was continued.

References:

1. Gómez Herrero H, Arraiza Sarasa M, Rubio Marco I, García de Eulate Martín-Moro I. Pulmonary rheumatoid nodules: presentation, methods, diagnosis and progression in reference to 5 cases. Reumatol Clin. 2012;8(4):​212-215.
2. Chhakchhuak CL, Khosravi M, Lohr KM. Role of 18F-FDG PET scan in rheumatoid lung nodule: case report and review of the literature. Case Rep Rheumatol. 2013;2013:621340. doi:10.1155/2013/621340

NEXT: Shrinking Lung Syndrome as a First Presentation of Systemic Lupus Erythematosus

Shrinking Lung Syndrome as a First Presentation of Systemic Lupus Erythematosus

Sherif A. Nasef, MD; Abdulaziz A. Nwasser Alheraiqi, MD; Mohammed Hashim K. Ali, MD; and Amer Zahralliyali, MD
King Fahad Specialist Hospital, Dammam, Saudi Arabia

Mutlaq Naheitan Alsubaie and Mohammad Abdullah Alutaibi
University of Dammam, Saudi Arabia

A 48-year-old woman was referred to our tertiary care center from a local hospital for a workup of unresolved pneumonia. She presented with a 6-month history of shortness of breath at rest, which was worsened by exertion and was associated with atypical chest pain. She had joint pain mainly involving the small joints, along with a photosensitive rash mainly on her face and bilateral swelling of the lower limbs.

History. Her past medical history was significant for hypertension, obstructive sleep apnea (for which she was on home continuous positive-airway pressure of 8 cm H₂O), and obesity (body mass index, 51 kg/m²). Her only current medication was salbutamol via metered-dose inhaler (MDI) as needed. Her social history was negative for smoking, alcohol use, and illicit drug use. Her family history was negative for pulmonary and autoimmune diseases.

Physical examination. On examination, the patient was afebrile, with a pulse of 76 beats/min, a blood pressure of 143/76 mm Hg, a respiratory rate of 20 breaths/min, and an oxygen saturation of 97% on room air.

Skin examination showed an erythematous malar rash. Cardiovascular examination findings were unremarkable. Chest examination showed fine bilateral basal crackles up to the mid chest with decreased breath sounds. Her abdomen was soft and nontender with no organomegaly or ascites. Her lower extremities showed 2+ pitting pedal edema. Examination of the joints showed no swelling or tenderness.

Diagnostic tests. Laboratory studies disclosed the following values: white blood cell (WBC) count, 5000/µL; hemoglobin, 11.8 g/dL; platelet count, 215 × 10³/µL; erythrocyte sedimentation rate, 35 mm/h; ferritin, 208 ng/mL; protein, 6 g/dL; and albumin, 2.2 g/dL. Results of renal function tests, thyroid function tests, and liver enzyme tests were normal. 

Urinalysis was negative for casts, WBCs, and red blood cells. The urine albumin level was 1620 mg/24 hr.

Antinuclear antibody test results were positive at a 1:1280 dilution with a homogeneous pattern; anti-DNA antibody test results were strongly positive at 1325 IU/mL; anti SS-A test results were strongly positive; anti SS-B test results were negative; and anti-RNP antibody test results were negative. The C3 level was 0.8 g/L (reference range, 0.91-2.41 g/L) and the C4 level was 0.07 g/L (reference range, 0.078-0.52 g/L). Direct Coombs test results were positive, anticardiolipin immunoglobulin G and A results were positive, but immunoglobulin M results were negative. β₂ glycoprotein I antibodies and lupus anticoagulants were negative.

Pulmonary function test (PFT) results showed a restrictive pattern, with a forced expiratory volume in the first second of expiration (FEV₁) of 0.77 L (30% of predicted), forced vital capacity (FVC) of 0.81 L (27.6% of predicted), FEV₁/FVC ratio of 94.6%, total lung capacity (TLC) of 1.9 L (40% of predicted), and carbon monoxide diffusing capacity (Dlco) of 1.35 mmol/min/kPa (17% of predicted).

Chest radiography showed bilateral atelectasis with bilateral pleural effusion, more so on the right side (Figure 1). High-resolution computed tomography (CT) scanning showed bibasilar airspace opacity suggestive of atelectasis, with pleural thickening consistent with pleural fibrosis, and with trace pleural effusions but no evidence of interstitial lung disease (Figure 2). CT angiography of the chest showed no evidence of pulmonary embolism. Echocardiography showed mild concentric left ventricular hypertrophy with a normal ejection fraction. No valvular abnormalities or pulmonary hypertension were noted.

She received a diagnosis of systemic lupus erythematosus (SLE) with lupus nephritis and shrinking lung syndrome (SLS).

Treatment. She was started on 1500 mg of mycophenolate mofetil twice a day, 60 mg of prednisone once a day, 200 mg of hydroxychloroquine twice a day, and 2 puffs of salbutamol via MDI 4 times a day. She was seen by a nephrologist, 160 mg of valsartan once a day was added to her regimen, and kidney biopsy was recommended. The patient improved on these medications, and her shortness of breath resolved. The prednisone dosage was tapered to 10 mg daily.

Outcome of the case. Three months later, a follow-up chest radiograph showed resolution of pleural effusions and decreased bibasilar atelectasis (Figure 3). PFT results at that time showed significant improvement, with FEV₁ of 1.02 L (41% of predicted), FVC of 1.25 L (42% of predicted), FEV₁/FVC ratio of 82%, TLC of 2.26 L (47.5% of predicted), and Dlco of 3.7 mmol/min/kPa (46.6% of predicted).

Discussion. SLS is a rare respiratory system manifestation associated with autoimmune diseases, particularly with SLE and rarely with Sjögren syndrome and polymyositis.¹

SLS was first described by Hoffbrand and Beck in 1965,² and it is characterized by reduction in lung volume, elevation of the diaphragm, and a restrictive pattern on PFTs with no parenchymal involvement. Patients usually present with dyspnea and chest pain.¹ Given that SLS is a rare manifestation of SLE, excluding other SLE-related pulmonary manifestations including pleural, parenchymal, and vascular lesions is important.¹

The pathogenesis of SLS remains unknown. When Hoffbrand and Beck first described the syndrome, they attributed it to the loss of surfactant leading to hyaline formation and microatelectasis of the alveoli. Other hypotheses suggest diaphragmatic weakness due to phrenic nerve neuropathy, pain-induced diaphragmatic inhibition, and dysfunction of the diaphragm due to corticosteroid therapy.^1,3 Electromyography studies of the phrenic nerve showed neither demyelination nor degeneration neuropathies causing diaphragmatic weakness.³ Duron and colleagues⁴ have suggested that pleural chest pain is an important symptom of SLS, which might be caused by pleural effusion or inflammation and will cause diaphragmatic immobility. The involvement of corticosteroids is unlikely given the fact that some patients present with SLS without previous corticosteroid treatment, and some patients show improvement after corticosteroid therapy.^1,3,4

Respiratory involvement in SLE patients is common and can include pulmonary fibrosis, pulmonary hypertension, pleural fibrosis, pulmonary infarction, and SLS. According to a cohort study done by Bertoli and colleagues,⁵ 4 of 626 SLE patients developed SLS, representing a prevalence of 0.6%.

Our patient was not known to have SLE until she presented with dyspnea and chest pain unresponsive to antibiotic therapy for presumed pneumonia. Her workup revealed the underlying SLE with kidney involvement. In such a setting, her chest radiographs showing bilateral atelectasis along with bilateral pleural effusion, more so on the right side, were suggestive of SLE. High-resolution CT ruled out parenchymal lung disease, while echocardiography and CT angiography ruled out pulmonary vascular disease, thus confirming the diagnosis of SLS. Treatment of her kidney involvement with corticosteroids and mycophenolate improved her chest radiograph findings and PFT abnormalities in a short period.

There is no standard treatment for SLS, but systemic corticosteroids are considered a first-line treatment. In corticosteroid-refractory cases, immunosuppressants can be used. Duron and colleagues⁴ treated 15 SLS patients with corticosteroids at high and low doses, then used cyclophosphamide in case of corticosteroids failure, but the regimen showed no efficacy. Patients treated with methotrexate alone showed mild improvement in PFT results.⁴ In another study, rituximab showed improvement in SLS after failure of systemic corticosteroid therapy.⁶

References:

1. Carmier D, Diot E, Diot P. Shrinking lung syndrome: recognition, pathophysiology and therapeutic strategy. Expert Rev Respir Med. 2011;5(1):33-39.
2. Hoffbrand BI, Beck ER. “Unexplained” dyspnoea and shrinking lungs in systemic lupus erythematosus. Br Med J. 1965;1(5445):1273-1277.
3. Singh R, Huang W, Menon Y, Espinoza LR. Shrinking lung syndrome in systemic lupus erythematosus and Sjogren’s syndrome. J Clin Rheumatol. 2002;8(6):340-345.
4. Duron L, Cohen-Aubart F, Diot E, et al. Shrinking lung syndrome associated with systemic lupus erythematosus: a multicenter collaborative study of 15 new cases and a review of the 155 cases in the literature focusing on treatment response and long-term outcomes. Autoimmun Rev. 2016;15(10):994-1000.
5. Bertoli AM, Vila LM, Apte M, et al; LUMINA Study Group. Systemic lupus erythematosus in a multiethnic US Cohort LUMINA XLVIII: factors predictive of pulmonary damage. Lupus. 2007;16(6):410-417.
6. Peñacoba Toribio P, Córica Albani ME, Mayos Pérez M, Rodríguez de la Serna A. Rituximab in the treatment of shrinking lung syndrome in systemic lupus erythematosus. Reumatol Clin. 2014;10(5):325-327.