Coexistence of Sjögren's syndrome and sarcoidosis: a report of five cases

Mansour, Mohammed; Al‐Hashimi, Ibtisam; Wright, John M.

doi:10.1111/j.1600-0714.2007.00530.x

Cited by 26 publications

(24 citation statements)

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“…to our knowledge, reports of a coexistence of both conditions with histological proof of sarcoidosis are relatively scarce [4][5][6][7]. Our case underlines how important pathology can be to establish the true coincidence of sarcoidosis with pSS.…”

Section: Discussionmentioning

confidence: 73%

“…If such classification criteria were taken as "the truth" in clinical practice, sarcoidosis and pSS would be regarded as mutually exclusive conditions and their coexistence regulated as "impossible" or at least "unlawful." however, the prevalence of sarcoidosis in large series of patients with pSS ranges from 1 to 2 % [4][5][6]. although classification criteria are not to be taken as diagnostic criteria, they are bound to affect clinicians' decisions and perceptions in clinical practice and induce diagnostic error or suboptimal management.…”

Section: Discussionmentioning

confidence: 99%

“…Both chronic inflammatory conditions of unknown origin are characterized by an intense cellular immune infiltrate at disease sites, predominantly composed of t lymphocytes [4,5]. the similarity of the underlying immunopathogenetic mechanisms has been argued as an explanation for the coexistence of both diseases.…”

Section: Discussionmentioning

confidence: 99%

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Coexisting primary Sjögren’s syndrome and sarcoidosis: coincidence, mutually exclusive conditions or syndrome?

Santiago

Rovisco

et al. 2014

Rheumatol Int

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the initial laboratory evaluation revealed an erythrocyte sedimentation rate (ESR) of 61 mm/h (normal value <10) and a serum C-reactive protein (CRP) level of 12 mg/L (normal value <5). Rheumatoid factor was 184 uI/mL (normal range <20), and serum angiotensin-converting enzyme (SaCE) was 65 u/I (normal value 8-52 u/L). Full blood count and blood chemistry were all within the normal range. Immunoglobulin G levels were 21.3 g/L (normal values 7.0-16.0 g/L); kappa light chain 18.58 g/L (6.66-14.65 g/L); and lambda light chain 7.43 g/L (2.99-6.99 g/L), with no detection of monoclonal component. Purified protein derivative test result was negative (an induration <5 mm).her chest radiograph (Fig. 1a) and high-resolution computed tomography (hRCt) (Fig. 2a) showed hilar and mediastinal lymphadenopathy and multiple small pulmonary nodules. Lung function tests and carbon monoxide diffusing capacity were normal. Bronchoalveolar lavage demonstrated an increase in CD4+/CD8+ t cell ratio (12.4). mycobacterial and fungal cultures were negative. to investigate the pulmonary nodules, a mediastinoscopydirected biopsy was performed. the histological exam revealed multiple noncaseating granulomas.the diagnosis of sarcoidosis (stage II) was established based on the clinical, imaging, and pathological findings. the patient was diagnosed with coexisting pSS and sarcoidosis.Because the respiratory symptoms and fatigue worsened over time, the patient was empirically treated with prednisolone 0.3 mg/kg/day for 6 weeks, with a slow tapering over 6 months, associated with calcium supplementation and alendronic acid/colecalciferol (70 mg/5,600 uI, weekly) [1].Gradually, all the symptoms improved, and 1 year later, the patient denied any respiratory or systemic symptoms.Abstract herein, we describe a 44-year-old female diagnosed with histologically proven coexistence of primary Sjögren's syndrome and sarcoidosis with pulmonary and muscular involvement. the differential diagnosis may be difficult, but this is not an exceptional case, which highlights the need to critically revise the consideration of sarcoidosis as an exclusion for primary Sjögren's syndrome, as established in current classification criteria.Keywords Primary Sjögren's syndrome · Sarcoidosis · Lung · muscle Case reporta 44-year-old Caucasian female was referred to our Department due to a 2-week history of dyspnea, persistent dry cough, arthralgia, and fatigue. Four years previously, primary Sjögren syndrome (pSS) had been diagnosed on the presence of sicca symptoms associated with SSa and SSB auto-antibodies, and a salivary gland biopsy demonstrating lymphocytic infiltrates (Chisholm-mason classification grade IV). She had been successfully treated with hydroxychloroquine 400 mg/day and an ophthalmic emulsion. her family and occupational and environmental histories were not contributory.Physical examination showed normal vital signs, and cardiovascular, pulmonary, and musculoskeletal clinical examinations were unremarkable. no peripheral adenopathies were noted.

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Section: Discussionmentioning

confidence: 73%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Coexisting primary Sjögren’s syndrome and sarcoidosis: coincidence, mutually exclusive conditions or syndrome?

Santiago

Rovisco

et al. 2014

Rheumatol Int

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“…They reported that they demonstrated sarcoidosis and Sjögren's association but noted sarcoidosis should not be present as an exclusion criterion in diagnosing SS. Mansour et al (2) and defined their accounting for the diagnosis criteria of both diseases as an association in their series of five cases and they made the SS diagnosis by using American-European criteria (at least one auto-antibody or accounting for four criteria out of six accompanying biopsy). They also pathologically demonstrated sarcoidosis diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Sarcoidosis Imitating Sjögren's Syndrome or the Association of Sarcoidosis - Sjögren's Syndrome

Diken¹,

Çiledağ²,

Atasoy³

et al. 2016

Respir Case Rep

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Sarcoidosis and Sjögren's syndrome are chronic multi-systemic diseases. The association of these two diseases was reported only in 1% of patients with Sjögren's syndrome. We present this rare case in order to draw the attention to the prognostic importance of this difference. A 62-year-old female patient presented to our hospital with the complaints of dyspnea and a dry cough. Dry eye and dry mouth were present. Laboratory analyses revealed serum anti SS-A (Ro) (+++) antibody positivity. Chest computerized tomography revealed lymphadenopathy and reticular-micronodular appearance evident. Transbronchial needle aspiration guided with endobronchial ultrasound was conducted through 10R and 7 lymph nodes. Pathology was compatible with sarcoidosis. Pulmonary function test revealed 40% carbon monoxide diffusion capacity. It is of prognostic importance to distinguish sarcoidosis imitating Sjögren's syndrome from the association of sarcoidosis-Sjögren's syndrome, since sarcoidosis mostly limits itself and recover by itself without any functional restrictions, but the pulmonary involvement of Sjögren's syndrome causes permanent defects.

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“…Luis et al presented two cases of sarcoidosis with lung involvement and Sjogren's Syndrome. In that cases, noncaseating granuloma and lymphocytic interstitial pneumonitis were identified in the lung pathology (23)(24)(25). Combined pulmonary sarcoidosis and lung involvement of Siogren's Syndrome may require lung biopsy to show noncaseating g r a n u l o m a s a n d lymphocityc interstitial pneumonitis.…”

Section: Sarcoidosis and Sjogren's Syndromementioning

confidence: 99%

Sarcoidosis and autoimmune disorders. A case report and review of the literature

Ozkan¹

2015

SOB

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SummarySarcoidosis is a multisystem disease of unknown etiology characterized by clusters of non-necrotizing granulomas and distortion of surrounding tissue. Although some patients are asymptomatic, sarcoidosis can cause a wide range of signs and symptoms often mimicking autoimmune rheumatologic disorders. Combined sarcoidosis and autoimmune disorders is uncommon as a result of either an University Hospital database analysis and literature review. However, given the high prevalence of autoimmunity in the general population, clinicians should be alerted to correctly diagnose and manage patients suspected to have both sarcoidosis and autoimmune disorders. KEY WORDS: sarcoidosis, autoimmunity, connective tissue disease. IntroductionSarcoidosis is a systemic inflammatory disorder that involves multiple tissues and is characterized by a non-caseating granulomatous reaction (1). Despite the etiology is not well understood, there are cellular immune system activation and nonspecific inflammatory response similar to some rheumatologic disease which are considered to play an important role in the pathogenesis (2). Sarcoidosis has been reported in all races and ethnic groups with a marked variations. People from different ages can be affected, but it is particularly sighted among the young adults whose ages range from 20 to 40, with possibly a second peak © C I C E d i z i o n i I n t e r n a z i o n a l i both legs together with the appearance of multiple skin papules. A skin biopsy revealed noncaseating epithelioid genulomas, consistent with the diagnosis of sarcoid granulomatosis dermatitis. Then, the patient started a treatment with oral prednisolone tablets (initially 0.75mg/kg body weight then tapering) showing a complete recovery of both skin lesions and leg sign and symptoms. Recognition of cutaneous sarcoid lesions was important in our patient because they provided a visible clue to the contribution of sarcoidosis for patient symptoms. Nevertheless, the diagnosis of cutaneous lesions in a patient with RA and sarcoidosis may be misleading because lesions can exhibit many different morphologies common to both diseases. Literature reviewSarcoidosis is a T helper 1 (Th1) mediated granulomatous disease, with unknown etiology, involving more than one organ. Sarcoidosis may mimic a number of rheumatic diseases or may coexist side by side. It can commonly present with clinical findings that are indicative of vasculitis and spondyloarthritis or connective tissue diseases, including both primary Sjogren's Syndrome, Systemic Lupus Erythematosus, rheumatoid arthritis, and sarcoidosis (1). Some constellations of clinical signs or symptoms are almost pathognomonic for sarcoidosis, including bilateral hilar nodal enlargement as an isolated clinical finding, erythema nodosum with bilateral hilar adenopathy (Lofgren Syndrome), and uveitis combined with fever plus parotiditis (Heerfordt Syndrome). Excluding Lofgren's and Heerfordt's Syndromes, the diagnosis of sarcoidosis requires a tissue biopsy confirming granulomatous inflammat...

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Coexistence of Sjögren's syndrome and sarcoidosis: a report of five cases

Abstract: Our findings appear to support a true coexistence of sarcoidosis with SS. Therefore, it is reasonable to suggest removing the exclusion of sarcoidosis from the diagnostic criteria for SS.

Cited by 26 publications

References 23 publications

Coexisting primary Sjögren’s syndrome and sarcoidosis: coincidence, mutually exclusive conditions or syndrome?

Coexisting primary Sjögren’s syndrome and sarcoidosis: coincidence, mutually exclusive conditions or syndrome?

Sarcoidosis Imitating Sjögren's Syndrome or the Association of Sarcoidosis - Sjögren's Syndrome

Sarcoidosis and autoimmune disorders. A case report and review of the literature

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