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Pulmonary and splenic sarcoidosis with atypical onset

Raluca Guteanu, Ionela Belaconi, Alina Croitoru, Andrei Cristian Bobocea, Miron Alexandru Bogdan

Abstract: 

Sarcoidosis is a multisystemic disorder that can mimic other pathologies with multiorgan involvement, with a difficult differential diagnosis and delaying the definitive diagnosis. Sarcoidosis can take atypical clinical and radiological forms, often being mistaken for tuberculosis or neoplasia. The authors present the case of a 46-year- old male patient who underwent splenectomy for the suspicion of a lymphoma. The histopathologic examination confirmed the diagnosis of sarcoidosis. After one year of treatment with methylprednisolone, significant regression of the pulmonary micronodular opacities and lymphadenopathies (thoracic and abdominal) was revealed, with serum angiotensin convertase (ACS) returned to normal and the patient stationary at the functional respiratory tests. The specificity of this case is the atypical onset of symptoms only in the abdominal area, without respiratory manifestations (dyspnea or cough), contrary to the involvement of the pulmonary interstitium, being necessary a splenectomy to establish the diagnosis.

 

Keywords: 
atypical sarcoidosis, lymphoma, splenectomy

Introduction

Sarcoidosis is an inflammatory systemic disease of unknown etiology characterized by the presence of non-caseating granulomas with the involvement of any organ(1). The primary location of the disease is the pul-monary system, in more than 90% of cases(2). Although abdominal involvement is uncommon, it can more easily mimic an infectious or a neoplastic pathology, causing real differential diagnosis issues(3).

Case presentation

The authors present the atypical case of a 46-year-old male patient with no previous pathology, describing the insidious onset of a left hypochondrium pain and facial hyperemia. The patient was referred to the hematology department where a whole-body computed tomography (CT) scan revealed bilateral micronodular pulmonary opacities (Figure 1A), mediastinal and abdominal lym-phadenopathies, significant spleen enlargement (Figure 1B), findings suggestive for non-Hodgkin malignant lymphoma. An osteomedullar biopsy was performed and showed the presence of normoblastic erythroblastic hyperplasia, possibly of neoplastic etiology.

The patient was referred to the department of gen-eral surgery for splenectomy and the pneumological preoperative assessment excluded pulmonary active infection with Mycobacterium tuberculosis.

A laparoscopic splenectomy and lymph node biopsy of the splenic hilum were performed, with initial unfa-vorable postoperative outcome and the onset of fever (38-39 °C) on day one.

A second chest and abdominal CT scan showed the presence of newly formed bilateral pulmonary consolida-tion areas, with bilateral pleural effusion, more on the left side (Figures 2A and 2B). Left chest tube drainage and broad-spectrum antibiotic therapy were initiated, with the remission of fever and the resolution of pulmo-nary radiologic elements of an infectious substrate (Figures 3A and 3B).

The histopathological and immunohistochemistry examinations of the removed spleen and splenic hilum lymph node revealed non-caseating chronic granuloma-tous inflammation, positive CD68, CD4, CD8, CD4/CD8 ratio = 5.2, suggestive features of sarcoidosis, but that cannot rule out tuberculosis. For a differential diagno-sis, polymerization chain reaction (PCR) from the patient’s sputum once again excluded the infection with Mycobacterium tuberculosis.

The patient was referred to our pneumology depart-ment for further investigations and treatment at 5 months from the onset of symptoms.

Bronchoscopy with bronchial-alveolar lavage showed the predominance of lymphocytes – 49%, CD4/CD8 ratio =4.1, and serum angiotensin convertase (ACS) of 67 U/L (laboratory normal values range 12-65 U/L). The res-piratory functional samples showed mild restrictive ventilatory dysfunction and normal alveolo-capillary diffusion. The 6-minute walking test was within normal parameters and without desaturation. All investigations supported the histopathological diagnosis of sarcoidosis (Table 1).

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Given the multisystemic involvement, with splenic, pulmonary and lymph nodes, the decision was to begin the therapy with methylprednisolone 32 mg per day, decreasing the dose progressively over a one-year period.

The patient was evaluated periodically, with thoracic and abdominal CT scans revealing significant regression of the pulmonary micronodular opacities and lymphad-enopathies (thoracic and abdominal) towards the end of treatment (Figures 4A and 4B).

ACS was converted to normal values, and the respira-tory functional tests showed stationary status. The follow-up at 12 months did not show signs of relapse (Table 1).

Discussion

The incidence of splenic sarcoidosis ranges between 10% and 50%, depending on the clinical judgement, imagistic findings and the histopathological examina-tion of various resected specimens(4).

Spleen involvement is mostly asymptomatic, but occasionally pain has been described in the left hipoc-ondrum, as well as non-specific symptoms such as sweating, fever and malaise.

In about 3% of patients with splenic sarcoidosis, mas-sive spleen enlargement was described. In isolated cases, hypersplenism causes various grades of anaemia leuko-penia, thrombocytopenia, to severe conditions including pancytopenia. The radiological findings of splenic sar-coidosis are sometimes multiple nodules throughout the parenchyma, that mimic abdominal carcinomatosis, a feature found more frequently in malignant lymphoma(5).

Most cases of splenic sarcoidosis do not require sur-gical treatment. The indications of splenectomy include abdominal pain due to splenomegaly, hypersplenism, functional asplenia or spontaneous splenic rupture(6).

The specificity of this case is the atypical onset of symptoms only in the abdominal area, with pain in the left hypochondrium, which is a general symptom, with-out pathognomonics of a particular disease. The patient didn’t present respiratory symptoms (dyspnea or cough), contrary to the rather important involvement of the pulmonary interstitium revealed at the thoracic CT examination. Functional respiratory samples, however, showed mild restrictive syndrome.

In this case, the laparoscopic splenectomy was per-formed due to the high suspicion of lymphoma, taking into account the initial histopathological result of the osteomedullar biopsy and the isolated abdominal symptoms.

Similar cases of splenic sarcoidosis are cited in the medical literature, proving the variability of the clinic forms of sarcoidosis(6).

 

References: 
  1. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007; 357(21):2153–2165.
  2. Wu JJ, Schiff KR. Sarcoidosis. Am Fam Physician. 2004; 70(2):312–322.
  3. Hammen I, Sherson DL, Davidsen JR. Systemic sarcoidosis mimicking malignant metastatic disease. European Clinical Respiratory Journal. 2015; 2:10.3402/ecrj.v2.26761.
  4. Judson MA. Extrapulmonary sarcoidosis. Semin Respir Crit Care Med. 2007; 28(1):83–101.
  5. Warshauer DM, Dumbleton SA, Molina PL, Yankaskas BC, Parker LA, Woosley JT. Abdominal CT findings in sarcoidosis: radiologic and clinical correlation. Radiology. 1994; 192(1):93–98.
  6. Sreelesh KP, Kumar MLA, Anoop TM. Primary splenic sarcoidosis. Proceedings (Baylor University Medical Center). 2014; 27(4):344-345.

 

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