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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 869-873
Evolution from "typical" to "atypical" radiological appearances- report of two patients of Sarcoidosis


Department of Cardiorespiratory Physiology, Clinical Research Centre, Vallabhbhai Patel Chest Institute, University of Delhi, Delhi - 110 007, India

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Date of Submission13-Jun-2005
Date of Acceptance10-Aug-2006
 

   Abstract 

Two patients of pulmonary sarcoidosis are reported in whom during the course of the disease, the radiological lesions evolved from the more common presentations to unusual ones. In a 70 years old man, the evolution occurred from Stage I (bilateral hilar lymphadenopathy, BHL) to Stage II with reticular pattern and BHL. This was accompanied by multiple large pulmonary nodules and pleural effusion. In the second patient, a 48-year-old woman, the picture evolved from a typical stage II (BHL with bilateral reticulonodular pattern) to a large mass-lesion simulating a malignancy. Both the patients had a variable symptom-free period between the two presentations. The diagnosis was confirmed by the demonstration of noncaseating granulomas by transbronchial biopsy and transthoracic fine needle aspiration cytology, respectively.

Keywords: Pulmonary Sarcoidosis, HRCT, Chest Xray

How to cite this article:
Chhabra S K, Chugh T, Chhabra P. Evolution from "typical" to "atypical" radiological appearances- report of two patients of Sarcoidosis. Indian J Radiol Imaging 2006;16:869-73

How to cite this URL:
Chhabra S K, Chugh T, Chhabra P. Evolution from "typical" to "atypical" radiological appearances- report of two patients of Sarcoidosis. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Dec 13];16:869-73. Available from: http://www.ijri.org/text.asp?2006/16/4/869/32371
Sarcoidosis is a multisystemic granulomatous disorder of unknown etiology most commonly affecting young adults (in their 3rd to 4th decade) with a variable female predominance. It presents most commonly with bilateral lymphadenopathy, pulmonary infiltration and skin or eye lesions [1]. Upto 30- 60% of patients with sarcoidosis are asymptomatic with incidental findings on chest radiograph [2]. While a wide range of radiological presentations have been described, the commonest is a bilateral hilar lymphadenopathy (BHL) with or without diffuse, bilateral, usually symmetrical reticulonodular shadows [3],[4],[5],[6]. This may be called a "typical" presentation. Less often, the radiological presentation may be with large nodular, alveolar, or mass-like opacities, mimicking other chest diseases including malignancy [5],[6],[7]. Such presentations may be called "atypical".

Most series of patients with sarcoidosis have described the different radiological appearances at the time of presentation as a cross-sectional study [3],[5],[7]. A few series and case reports have documented progression of disease but with "typical" pulmonary appearances [4],[5],[8],[9]. Evolution from a "typical" picture to an "atypical" one during the natural course of the disease has not been documented. We report two such cases.

Case I

A 70 years old non-smoker man presented to the outpatient clinic at the Institute in 1994, with complaints of dry cough for 10 days. A plain chest PA radiograph (Fig 1) revealed BHL. Mantoux test was negative. Lung function test (spirometry) was within normal limits. The cough resolved with symptomatic treatment and in the absence of any other symptoms and clue, a diagnosis of stage I sarcoidosis was made. Serial plain chest radiographs in 1996, 1999 and in 2000 did not reveal any change and the patient remained asymptomatic. He presented to us again in April 2004 with complaints of gradually progressive exertional breathlessness, without wheeze, and with dry cough for about 3 years. Initially, he attributed his breathlessness to old age but when it increased from Medical Research Council grade1 to 3, he sought medical advice. There was no history of fever, weakness, anorexia, weight loss, joint pains, and eye or skin involvement. Physical examination was unremarkable.

Preliminary investigations revealed the following: Hb-13.5gm %, TLC- 6750/mm3, DLC- P73 L15 E4 M3, ESR-106 mm in 1st hr, platelets count-1.95 lac/mm3, blood sugar (fasting)- 87 mg%, blood urea- 35.3mg%, serum creatinine-1.0 mg%, serum calcium 9 mg%, urine examination for proteins and sugar - negative, urine microscopic examination - normal. The Mantoux test (1 TU) site did not show any induration. The Chest PA radiograph revealed BHL with parenchymal involvement consisting of irregular bilateral mid and lower zone reticulation and several fairly well-defined round opacities, 1 to 3 cm scattered in the right mid zone and left lower zone (Fig 2). In view of the past history and the current radiological appearances, a diagnosis of sarcoidosis was made with a possibility of secondary metastases to the lung.

A high-resolution CT scan of the chest revealed BHL with right paratracheal lymphadenopathy and irregular lower and middle zone bilateral reticulation, peribronchial thickening, areas of ground glass haze, and several fairly well-defined round opacities, 1 to 3 cm scattered in right upper and both lower zones. A small pleural effusion was also seen. (Fig 3a, and Fig 3b). The effusion was too small to be tapped. On further investigation, the serum angiotensin converting enzyme (SACE) levels were found to be raised to 80 IU/L (normal less than 52 IU/L) while the 24-hrs urinary calcium excretion was normal at 95 mg% (normal upto 200mg%). The antinuclear and the rheumatoid factor titres were normal. The pulmonary function tests revealed a mixed restrictive-cum-obstructive pattern with impaired diffusion. The values were: forced vital capacity (FVC)- 2.42 L (69% predicted), forced expiratory volume in 1 sec (FEV1)-1.18 L (42% predicted), FEV1/ FVC- 49 (62% predicted), residual volume (RV)- 2.28L (100% predicted), total lung capacity (TLC)- 4.7 (81% predicted), single breath carbon monoxide diffusion capacity DLCOSB-10.39 (43% predicted), and KCO (DLco/VA)- 3.23 (83% predicted). There was no remarkable change in spirometric values after administration of inhaled salbutamol. The ultrasound examination of abdomen was unremarkable. Fibreoptic bronchoscopy did not show any abnormal appearances while a transbronchial lung biopsy showed multiple discrete non-caseating granulomas consisting of epithelioid cells and multinucleate giant cells consistent with sarcoidosis (Fig 4).

The patient was advised oral prednisolone, 1 mg/Kg/day. At follow-up at 2 months, he reported substantial improvement in symptoms. However, the lung function was unchanged with the FVC of 2.48 L and the FEV1 of.19 L. The chest radiograph also did not show any remarkable change. The patient is currently being followed up.

Case II

A 48 years old non-smoker woman patient presented with cough associated with minimal mucoid expectoration and progressively increasing breathlessness on exertion for the last 2 years. She had received symptomatic treatment but a diagnosis had not been made. There was no other remarkable feature in history. Physical examination including chest did not reveal any abnormal sign.

Preliminary investigations showed: Hb 11.6g%, TLC - 9200/mm3, DLC - P70 L26 M2 E2. Sputum smear examination was negative for acid- fast bacilli. Mantoux test with 1 TU revealed no induration at 48 hours. The Chest PA radiograph showed bilateral hilar prominence with basilar reticular shadows. SACE level was increased to 126 IU/L There was no hypercalciuria. The pulmonary function tests showed a mixed restrictive-cum-obstructive pattern with impaired diffusion. The values were: FVC 1.30L (50% predicted), FEV1 0.82L (37% of predicted), TLC 3.6L (80% predicted) and RV 1.92L (120% of predicted). The DLCOSB was 11.82 (53% predicted) and KCO 5.65 (109% predicted). The computed tomograph (CT) of chest revealed bilateral prevascular, bilateral hilar and aortopulmonary lymph nodes enlargement with ground glass infiltration and basilar nodular shadows.

As the patient refused to undergo any invasive intervention (transbronchial lung biopsy/open lung biopsy) to confirm the diagnosis, a provisional diagnosis of sarcoidosis (Stage II) was made based on the above clinical, laboratory and physiological features. She was advised to take oral prednisolone, 1 mg/Kg/day. Regular monthly follow-ups were maintained with clinical examination and spirometry. At six months of regular treatment, there was a marked clinical and physiological improvement. The FVC increased to 1.92L (70% predicted). There was however not much change in the follow-up radiographs. The dose of prednisolone was gradually tapered off.

The patient remained asymptomatic for the next 12 months when she developed gradually increasing exertional breathlessness with cough and minimal expectoration. The plain Chest PA radiograph now showed an ill-defined heterogeneous opacity in the left mid-zone with increased hilar prominence and increased bilateral basal reticulations (Fig 5). Another CT scan was advised that revealed bilateral reticular-nodular opacities in pulmonary parenchyma, calcified mediastinal lymph nodes and a mass lesion in the left lower lobe with irregular margins and small air spaces in-between. The Mass extended from the left hilum to the pleural surface (Fig 6). Sputum gram stain and culture for pyogenic organisms, acid-fast bacilli and cytology for malignant cells were negative. Mantoux test also remained negative. A diagnosis of lung carcinoma was suspected with tuberculosis or pneumonia as the other possibilities. A fine needle aspiration was carried out under CT guidance from the mass lesion. Cytological examination revealed several noncaseating granulomas. The smears stained negative for mycobacterium and there was no evidence of malignant cells. The patient has been advised to start oral prednisolone (30 mg/day).


   Discussion Top


The chest radiological presentations in sarcoidosis can take several forms. The chest radiograph is abnormal in 90-95% of patients with sarcoidosis [2],[3],[4],[5],[6]. The most common abnormalities encountered are lymph node enlargement (hilar, tracheobronchial and paratracheal) and parenchymal lung involvement.

The traditional staging [1] of sarcoidosis is based on plain radiographic appearances and is classified as: stage 0 (normal chest radiograph); stage I (bilateral hilar lymphadenopathy, BHL); stage II (BHL associated with pulmonary infiltrations); stage III (pulmonary infiltrations without BHL); and stage IV (Pulmonary fibrosis). Although the feature of BHL has been emphasized, unilateral hilar lymphadenopathy (UHL), has also been reported [3]. The pulmonary involvement in stage II and III has been described as "infiltration" or "densities" and most commonly is a picture of diffuse, bilateral, usually symmetrical, reticulonodular shadows [4],[5]. Unilateral parenchymal infiltrates are uncommon [6]. The reticulonodular pattern consists of fine linear densities and small nodules measuring 3 to 5mm in diameter and may be pure nodular, pure reticular and mixed reticulonodular. It was observed in 74% of patients who had parenchymal lesions [5],[6]. This appearance may therefore be described as "typical". However, the pulmonary involvement may sometimes be 'atypical' ranging from miliary or micronodular, small nodular (less than 1 cm) or acinar shadows and rarely may even present as a consolidation, atelectasis or fibrocyctic disease or even as mass, thus simulating other pulmonary diseases including tuberculosis, asbestosis, vasculitis, carcinoma and fungal infections [5],[6],[7]. One case report of "Fairy Rings" in sarcoidosis has recently been described [10]. Stages I and II are seen at presentation in 75- 90% of patients in the west [5],[9] but studies from India have shown that stages II and III are more common, probably because of delayed diagnosis [3].

While cross-sectional series of patients highlighting the radiological diversity have been reported [3],[7], longitudinal studies on the course of the disease are fewer. In 70-80% of cases, lymph nodes with or without parenchymal lesions regress and eventually show radiographic resolution without residua [7],[8],[9]. On the other hand, progression from stage I to II has also been documented in previous studies [4],[6]. A study from India [4] showed that out of 43 patients of sarcoidosis studied, 21 had progressed from stage I to stage II before presentation as revealed from their records. Parenchymal lesions were described in this study as diffuse interstitial parenchymal fibrosis. In another study from New York [6], 22 of the 135 patients studied had progressed from stage I to stage II. Parenchymal lesions, seen in 81 patients (including 22 who had progressed) were divided into four subtypes (reticulonodular, miliary, nodular, large nodular or confluent densities), but there was no mention of evolution of type of parenchymal lesions in these 22 patients.

Large nodular opacities (with an average diameter of 1cm or more) are uncommon in sarcoidosis. These were seen only in 3 of the 95 patients with parenchymal lesions in a study by Kirks et al [5]. In the same study [5], during follow up, more than 60% of the patients developed parenchymal lesions. This included appearance or resolution of the commonest pattern (reticulonodular), resolution of pleural effusion or acinar shadows and the development of nodal calcification. In another study [6] large nodular and confluent densities (consolidation) were seen in 17 of 81 (21%) patients with parenchymal lesions. These nodular opacities may simulate metastatic neoplasms. The cases reported in the present study show that radiological appearances may change from the common "typical" patterns to the unusual, "atypical" patterns during the course of the disease. This was not observed in the previous studies on progress of the disease [4],[5],[6].

Another unusual observation in one of our cases was the presence of a pleural effusion. Pleural involvement in sarcoidosis is infrequent and reported with varying frequency of 0 to 7% [1],[2],[3],[5],[7],[9]. Pleural thickening, probably due to subpleural granulomas and pleural-based lesions due to conglomeration of these granulomas as seen on computed tomograms [11] is probably more common than a frank effusion.

While atypical and uncommon radiological appearances have been well described, the present study documents that the presentation may evolve from typical to atypical in a previously diagnosed patient of sarcoidosis. This is important to recognize as it usually prompts a search for another etiology. Undoubtedly, other causes of such "atypical" presentations should be investigated, but the possibility of these being due to sarcoidosis should also be considered. A histopathological examination of material from the lesion will usually provide the answer.

 
   References Top

1.American Thoracic Society, European Respiratory Society, World Association of Sarcoidosis and Other Granulomatous Disorders. Statement on sarcoidosis. Am J Respir Crit Care Med 1999; 160: 736-755.   Back to cited text no. 1    
2.Elias JA, Tanoue LT. Systemic sarcoidosis. In: Baum GL, Crapo JD, Celli BR, Karlinsky JB, eds. Textbook of Pulmonary Diseases, 6th ed. Philadelphia: Lippincott-Raven, 1998: 407- 430.   Back to cited text no. 2    
3.Gupta SK, Mitra K, Chatterjee S, Chakravarty SC. Sarcoidosis in India. Br J Dis Chest 1985; 79: 275-283.  Back to cited text no. 3  [PUBMED]  
4.Govindarajan R, Shah JR. Pulmonary sarcoidosis. J Assoc Phys India 1985; 33: 325-331.  Back to cited text no. 4    
5.Kirks DR, McCormick VD, Greenspan RH. Pulmonary sarcoidosis: Roentgenologic analysis of 150 patients. Am J Roentgenol 1973; 117: 777-786.  Back to cited text no. 5    
6.Ellis K, Renthal G. Pulmonary sarcoidosis: roentgenographic observations on course of disease. Am J Roentgenol 1962; 88: 1070-1083.  Back to cited text no. 6    
7.Rabinowitz JG, Ulreich S, Soriano C. The usual unusual manifestation of sarcoidosis and the "hilar haze" - a new diagnostic aid. Am J Roentgenol 1974; 120: 821-831  Back to cited text no. 7    
8.Scadding JG. Prognosis of intrathoracic sarcoidosis in England: a review of 136 cases after five years' observation. Br Med J 1961; 2: 1165-1172.  Back to cited text no. 8  [PUBMED]  
9.Fraser RG, Peter Pare JA, Fraser RS, Genereux GP. Diagnosis of diseases of the chest. 3rd ed. Philadelphia: W.B. Saunders, 1991: 2604-2647.   Back to cited text no. 9    
10.Marlow TJ, Krapiva PI, Schabel SI, Judson MA. The "Fairy Ring": a new radiographic finding in sarcoidosis. Chest 1999; 115: 275-276.   Back to cited text no. 10  [PUBMED]  [FULLTEXT]
11.Muller NL. Kullnig P, Miller RR. The CT findings of pulmonary fibrosis: analysis of 25 patients. Am J Roentgenol 1989; 152: 1179-1182  Back to cited text no. 11    

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Correspondence Address:
S K Chhabra
Department of Cardiorespiratory Physiology, Vallabhbhai Patel Chest Institute, University of Delhi, Delhi - 110 007
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32371

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  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]



 

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