Pulmonary Function Tests In Patients with Systemic Lupus Erythematosus (SLE)

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1 Pulmonary Function Tests In Patients with Systemic Lupus Erythematosus (SLE) Samiha Samuel, Mona Mohsen, Rasha M. Gamal El-Din, and Hossam Hosny 2 From the Departments of Pediatrics, Children s Hospital, Cairo University and Chest, 2 Cairo University, Egypt Abstract: Systemic lupus erythematosus (SLE) is an autoimmune disorder which primarily affects females. It may affect virtually any organ. Abnormalities of pulmonary function have been found in children with SLE even in absence of clinical or radiological evidence of pulmonary involvement. It is unknown whether these abnormalities represent an early sign of progressive lung disease or whether they associated with disease activity. The aim of our study was to investigate the frequency of pulmonary involvement in childhood SLE and to find the relationship between pulmonary function tests and other parameters of the disease. Our study included 7 patients with SLE; pulmonary manifestations were present in 36 patients with a percentage of.%. All patients with pulmonary affection whether clinical or radiological were subjected to pulmonary function tests (PFT). These included forced vital capacity and forced expiratory volume during first second. Among those 36 patients, restrictive pulmonary function was present in 22 patients (3.%). A significant statistical relation was detected between pulmonary function and C3 only (p value.%). Conclusion: Abnormalities of pulmonary function may be found in children with SLE even in absence of radiological evidence of pulmonary involvement and it is not related to other parameters of disease activity. So, it is recommended to perform pulmonary function tests even in absence of radiological evidence of pulmonary involvement to establish a diagnosis and aid long term follow up of SLE patients with acute lupus pneumonitis and diffuse interstitial lung disease which has a major impact on the mortality and morbidity of SLE patients. Studies to demonstrate the efficacy of PFT in diagnosing pulmonary involvement in SLE patients even in absence of clinical pulmonary manifestations are needed. Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disorder which primarily affects females. It may affect virtually any organ. Predominant manifestations include non-deforming arthritis, serositis, photosensitivity, renal, hematological and central nervous system involvement. Pleuritis occurs in 7-6% of patients at some point in the course of the disease. Although parenchymal lung disease is uncommon, pulmonary complications of SLE are potent and include acute lupus pneumonitis, diaphragmatic dysfunction and shrinking lung syndrome, cavitating pulmonary nodules, pulmonary hypertension, pulmonary vasculitis, pulmonary embolism, chronic interstitial pneumonitis, bronchiolitis obliterans and opportunistic pulmonary infections or drug toxicity from immunosuppressive therapy. 2 Abnormalities of pulmonary function have been found in children with SLE even in absence of clinical or radiological evidence of pulmonary involvement. It is unknown whether these abnormalities represent an early sign of progressive lung disease or whether they associated with disease activity. 3 The aim of this work is to investigate the frequency of pulmonary involvement in children with SLE and to determine the relationship between pulmonary function tests and other parameters of the disease. Subjects and Methods: Our study included 7 patients with SLE admitted to the inpatient wards of Children s Hospital, Cairo University, or sent for pulmonary function tests in the Chest Department, Cairo University, during the period from June 2 to January 2. They all presented before 3 years of age with a mean age of 3.3 ± 3. years. Our patients included 9 females and males. All patients fulfilled four or more of SLE criteria of the American College of Rheumatology. All patients were subjected to the following:. Detailed medical history including age, sex, age of onset, duration of illness and various clinical presentations especially pulmonary manifestations (e.g. cough, chest pain, dyspnea and cyanosis). Alexandria Journal of Pediatrics, Volume 9, Number 2, July 2 277

2 2. Thorough clinical examination with special attention to chest examination. 3. Laboratory investigations including complete blood picture, ESR, CRP, C3 and C levels, assessment of antinuclear antibodies (ANA), blood urea and serum creatinine, complete urine analysis, 2 hours urinary proteins,. Plain X-ray chest, CT scan of the chest if needed.. Pulmonary function tests in patients with pulmonary affection whether clinical or radiological and they included forced vital capacity and forced expiratory volume during first second. Results: Our study included 7 patients with SLE admitted to the inpatient wards of Children s Hospital, Cairo University or sent for pulmonary function tests in the Chest Department, Cairo University. Their age ranged from to 2 years with a mean of 3.3 ± 3. years; 8.3% were females (n=9) and.7% were males (fig.). Table I shows different clinical presentations and some laboratory data of SLE patients, where musculoskeletal manifestations were the commonest (9.3%), then alopecia in 8.3% and rash in 82.9% of patients. Regarding the results of CRP and ANA, ANA was positive in 9.7% of patients and CRP in 8.6% of patients. Table II: Patients demographic and laboratory data Data Mean S.D. Median Minimum Maximum Age Ill Duration Onset Age HB PLT TLC Lymph Staff Seg ESR C C Creat PTN2h Pus-Ur RBCs-Ur Female 8% 3 2 Male 6% 2 Fig. : Sex distribution among studied group Table I: Patients clinical presentations and laboratory data Data Frequency % Sex Male Female 9 (.7) (8.3) CRP 3 36 (8.6) (.) ANA 67 3 (9.7) (.3) Rash 8 2 (82.9) (7.) Photosensitivity 9 (8.3) (.7) Alopecia 6 (92.9) (7.) Muscloskeletal 66 (9.3) (.7) Arthritis 3 27 (6.) (38.6) Serositis 6 (22.9) (77.) Renal 6 (77.) (22.9) Cardiac 3 3 () () CNS (7.) (2.9) Pulmonary 36 3 (.) (8.6) PF Normal Restrictive 22 (3.) (2.) Dry cough Productive cough Dyspnea at rest Orthopnea Exersional dyspnea Chest pain Fig. 2: Pulmonary manifestations among studied group Hemoptysis Table II shows patient the demographic and laboratory data. Figure 2 shows pulmonary manifestations among the studied group, where the commonest were exertional dyspnea, productive cough and chest pain Tachypnea Abnormal chest shape Tracheal shift Dullness on percussion Diminished breath sounds Bronchial breathing Ronchi Crepitations Fig. 3: Pulmonary examination among studied group Alex J Pediatr, 9(2), July 2 278

3 Table III: Relation between PF with sex, CRP, ANA, And clinical presentation in SLE patients Data Sex Male Female CRP ANA Rash Photosensitivity Alopecia Muscloskeletal Arthritis Serositis Renal Cardiac CNS Normal Restrictive P Freq % Freq % value Table IV: Relation between PF with some demographic and laboratory data in SLE patients Data Normal Restrictive P (n=22) (n=) value Mean SD Mean SD Age Duration of illness Age at onset HB PLT TLC Lymph Staff SEG ERS C * C Creatinine PTN 2 h Pus in urine RBCs in urine No. of Cases Pulmonary manifestations Pulmonary functions Fig. : Pulmonary manifestations and pulmonary functions among studied group Consolidation X-ray finding Interstitial thic kening Pleural effusion Atelectasis pneumonic patches CT finding Ple ura l effusion Fig. :X-ray and CT findings among studied group The results of pulmonary examination are shown in figure 3, where the commonest were crepitations, tachypnea, ronchi and dullness on percussion. The results of pulmonary function are presented in figure, where restrictive pattern (positive finding) was present in patients (2%) and normal function in 22 patients (3.%). Figure shows different X-ray chest and CT chest findings among studied patients. Tables III and IV show the relation between PFT with sex, CRP, ANA, different clinical presentations of patients, some demographic and laboratory data where all showed a non significant relationship except for C3 only that showed a significant relationship. Discussion: Pulmonary abnormalities have been demonstrated in all collagen diseases. The early demonstration of lung involvement in SLE patients is difficult. The pulmonary manifestations in SLE vary from patient to patient but usually include cough with or without sputum, dyspnea, hemoptysis and chest pain. SLE may affect all the components of the respiratory system, including upper airways, lung parenchyma, pulmonary vasculature, pleura and respiratory muscles. 6 Pleuritis and pulmonary infections are the most prevalent pulmonary manifestations in SLE. Other associations include acute lupus pneumonitis, pulmonary hemorrhage, pulmonary vasculitis, pulmonary embolism and shrinking lung syndrome. The early detection of these diseases may help in preventing their fatal complications. In our work, the age of the patients ranged from to 2 years with a mean of 3.3 ± 3.. This was comparable to the mean age in the study by Al-Abbad et al., 8 in 2, which was to 6 years with a mean of 2 years. There was significant female predominance in our study with females representing 8.3% of our patients. This coincides with other studies done by Al- Abbad et al., 8 in Alex J Pediatr, 9(2), July 2

4 The illness duration in our study ranged from to 2 years with a mean of.2 ±2.89 years, which was more than that previously reported by Al-Abbad et al., 8 in 2, who recorded a mean of.9 years and was comparable with Galapatty et al., 9 in 2, who reported a duration of illness of 7 years. The most common clinical manifestations in our study were musculoskeletal manifestations in 9.3% of cases followed by alopecia 92.9%, photosensitivity in 8.3% and rash in 82.9% of patients. These results were coincident with Galapatty et al., 9 in 2, who reported that the commonest manifestations of SLE were mucocutaneous features in 98% and alopecia in 87%. Renal manifestations are defined clinically as mg or more proteinuria per 2 hours and or microscopic hematuria > RBCs/HPF or cellular casts on at least 2 occasions and/or serum creatinine greater than. mg/dl. We found renal manifestations in 77.% of our patients, and this is coincident with the studies by Cameron, in 99, who reported that clinically evident nephritis occurs in at least 7% of children with SLE. CNS manifestations were detected in 7.% of our patients and this figure was close to the study done by Steinlin et al., in 99, where the percent of CNS involvement were %. Pulmonary manifestations occurred in 36 patients with a percentage.%. The commonest was exertional dyspnea in 27 patients (38.%), productive cough in 2 patients (3.2%) and chest pain in 22 patients (3.%). These figures were less than those reported by Delgado et al., 2 in 99, who performed a study on 22 patients with SLE, 77% had respiratory symptoms including cough, chest pain, dyspnea and orthopnea. The most common findings on chest examination was crepitations in 9 patients (27.%), tachypnea in patients (2.%), diminished breath sounds and ronchi in patients (2%). Our results were in agreement with the results reported by Zamora et al., 3 in 997, who reported that tachypnea, cough, rales and dyspnea are frequently present. Chest X-ray was done for the 36 patients with pulmonary manifestations. It revealed interstitial thickening in patients (7.%), consolidation and pleural effusion, each in 2 patients (2.8%). These results showed that SLE patients with lung manifestations may have normal chest radiograph, as the sensitivity of chest radiographs in diagnosing pulmonary involvement in SLE is unsatisfactory. CT chest performed to patients with chest manifestations showed pneumonatic patches in 7 patients (%), atelectasis in patients (7.%) and pleural effusion in 2 patients (2.8%). Cheema and Quismorio, in 2, reported that high resolution computed tomographic scans of the chest and pulmonary function tests help to establish a diagnosis and aid long term follow up of SLE patients with acute lupus pneumonitis and diffuse interstitial lung disease which has a major impact on the mortality and morbidity of SLE patients. Pulmonary function tests were done for patients with pulmonary manifestations and it showed restrictive pattern in patients (2%) and normal pattern in 22 patients (3.%). These results were similar to those detected by Karim et al., 6 in 22, where lung volumes were reduced on pulmonary function tests in restrictive pattern. There were a non significant correlation between pulmonary function tests and all other parameters of the disease except for C3 level and this coincides with other studies which found that laboratory abnormalities such as low C3 or high ESR to be predictor of infections. 7 The definition of disease activity (flare) was the presence of new clinical manifestations and/or disease worsening compared with the previous evolution. 8 Our results were in agreement with those of Nakano et al., 9 in 22, who reported that there was no significant correlation between impairment of pulmonary function and the disease activity of SLE. Also, Sant et al., 2 in 997, mentioned that lung involvement can occur in the absence of activity in SLE. Conclusion & Recommendation: Abnormalities of pulmonary function may be found in children with SLE even in absence of radiological evidence of pulmonary involvement and it is not related to other parameters of disease activity. So, it is recommended to perform pulmonary function tests even in absence of radiological evidence of pulmonary involvement to establish a diagnosis and aid long term follow up of SLE patients with acute lupus pneumonitis and diffuse interstitial lung disease which has a major impact on the mortality and morbidity of SLE patients. Studies to demonstrate the efficacy of PFT in diagnosing pulmonary involvement in SLE patients even in absence of clinical pulmonary manifestations are needed. Alex J Pediatr, 9(2), July 2 28

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