Sarcoïdose musculaire : à propos d’un cas avec atteinte des muscles et des fascias et revue de la littérature

Introduction

Muscular involvement in sarcoidosis is noted in 50 to 80% of the patients when systematic muscle biopsies are carried out. Nevertheless, symptomatic forms are rare and three different types can be distinguished: nodular, chronic myopathy and acute myositis.

Case report

We report a 43-year-old man who presented with sarcoidosis involving in muscles, fascia and articulations. The diagnosis of sarcoidosis was supported by magnetic resonance imaging and positron emission tomography imaging and confirmed by a muscle biopsy. Clinical manifestations resolved with corticosteroids and methotrexate.

Conclusion

We review 23 cases of sarcoidosis myosistis most similar to ours. The commonest presentation is the involvement of the proximal limb muscles in association with elevated serum muscle enzymes.     

Sarcoïdose vertébrale. Évolution spontanément favorable : une observation et revue de la littérature

Introduction

The prevalence of vertebral sarcoidosis is highly variable (1 to 36% of reported case series). Because of limited clinical expression, its frequency is probably underestimated. Its proper management is not clearly defined.

Case report

A 42-year-old woman who had a past medical history of cutaneous and pulmonary sarcoidosis presented with low back pain that was refractory to usual medical treatment. A diagnosis of vertebral localisation of sarcoidosis was considered on the history of proven sarcoidosis, radiological features, and the absence of evidence of an alternative diagnosis. In the absence of other clinical or biological evidence of active sarcoidosis, a simple follow-up was planned. MRI control at 1 year showed the resolution of vertebral sarcoidosis lesions.

Conclusion

Spontaneous regression is a possible outcome of vertebral sarcoidosis. Initiation of a specific treatment should be discussed in the absence of other visceral involvement.     

Sarcoïdose médiastinale dans un contexte oncologique : apport de la ponction aspiration guidée par échoendoscopie

Introduction

Sarcoidosis and sarcoid reactions have been previously reported in association with cancer. Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) is a minimally invasive test for investigating mediastinal lymph nodes

Patients and methods

We conducted a retrospective review of 54 patients undergoing EUS-FNA in a cancer institute for suspected metastatic mediastinal lymph nodes showed by CT-imaging or positron emission tomography (PET). Patients with non-caseating granuloma identified by EUS-FNA were included

Results

EUS-FNA identified non-caseating granuloma in seven out of the 54 included patients. Most of them had positive PET. One patient had a prior history of sarcoidosis before the diagnosis of cancer. Another patient developed micrometastasis associated with sarcoid-like reaction. There was no adverse outcome associated with the EUS-FNA procedure

Conclusions

Sarcoidosis must be included in the differential diagnosis of patients with a history of malignancy who develop mediastinal lymphadenopathy. EUS-FNA is a safe and minimally invasive test to obtain tissue diagnosis     

Amylose AA compliquant une sarcoïdose : deux observations et revue de la littérature

Introduction

Sarcoidosis is a relatively common granulomatosis. Its outcome is usually favourable. AA amyloidosis is a rare complication of sarcoidosis. We report two new cases and review the literature.

Case reports

A 40-year-old woman followed for a splenomegaly was admitted for assessment of hepatic cholestasis. The diagnosis of sarcoidosis involving lungs, liver, spleen and lymph nodes was obtained on clinical, biological and histological evidence. Simultaneously, AA amyloidosis deposits was demonstrated on lymph nodes and spleen. The second patient was a 42-year-old woman who was evaluated for mediastinal lymph nodes and interstitial pneumonia. Lung biopsy showed epithelioid and giant cell granuloma without caseous necrosis, associated to AA amyloidosis deposits. In these two patients, corticosteroids (initially at 1 mg/kg daily) led to a sustained improvement with a 1 and 5 years follow-up, respectively.

Conclusion

AA amyloidosis is an uncommon complication of sarcoidosis. Corticosteroids associated with colchicine is proposed to treat simultaneously the two disorders.     

Sarcoïdose systémique au décours d’un traitement par interféron-alpha pégylé

A few cases of sarcoidosis have been reported in the course of chronic hepatitis C, with or without alpha-interferon treatment. We report a 54-year-old woman who presented a typical sarcoidosis in this context. The sarcoidosis involved skin, lungs and eyes. There are data in the literature supporting a pathogenic link between alpha-interferon and sarcoidosis: alpha-interferon possessing a non-specific immunologic inductor. The treatment of the sarcoidosis in this context is difficult and has to be adjusted to both the severity of the sarcoidosis and the viral hepatitis. Although sarcoidosis induced by alpha-interferon is rare, clinicians must be aware of this association.     

Uvéites révélant une sarcoïdose : caractéristiques cliniques à propos de 23 cas

Purpose

Uveitis may rarely reveal sarcoidosis in Caucasian patients. Our objective was to analyze the clinical manifestations, and the outcome in a group of patients in whom uveitis was the presenting manifestation of sarcoidosis.

Methods

Retrospective study including 23 patients (mean age: 50.3 ± 14.5 years) diagnosed with sarcoidosis after an episode of uveitis. Granulomatous lesions were documented in 14 patients.

Results

Ophthalmological examination revealed anterior uveitis (n = 5), intermediate uveitis (n = 2), posterior uveitis (n = 25) and panuveitis (n = 11). Ocular inflammation was bilateral in 16 patients (69,6%), typical aspects of granulomatous uveitis were found in only 16 eyes over 39 (41%), posterior uveitis was found in 18 eyes (46.2%), with an averaged visual acuity of 5/10. Macular oedema was noted in five patients. Suggestive signs of ocular sarcoidosis were present in 43% of the patients. Stage 1 or 2 pulmonary involvement (n = 22), musculoskeletal (22%), skin (13%), or spleen (9%) involvements were the most common findings. Oral corticosteroids were necessary in 91.3% of the patients, immunosuppressive agents in 26.1%, with a prolonged treatment greater than two years in 58%. The visual prognosis was good, with visual acuity greater than 6/10 in 96% of the cases if the ocular inflammation spared retina and choroid. However, a visual acuity less than 6/10 was observed in 44% of the cases when the posterior segment was involved.

Conclusion

Sarcoidosis may be revealed by an intraocular inflammation, with typical patterns in only 43% of the cases. Sarcoidosis should therefore be included in the differential diagnosis of every uveitis. Oral corticosteroids are required in almost all cases, owing to ocular involvement rather than visceral involvement.     

Nasosinusal and cervical sarcoidosis: a case series of three patients and literature review

Introduction

Sinonasal sarcoidosis is difficult to treat. Infliximab seems to be useful in the treatment of sarcoidosis of the upper respiratory tract.

Case series

We report three cases of sinonasal sarcoidosis in two women of 36 and 42-year-old and in a 64-year-old man. Resistance or dependence to corticosteroids and absence of efficacy of methotrexate therapy in one patient led to administer anti-TNFα therapy with infliximab. Outcome was favourable on sarcoid lesions but treatment was discontinued because of infectious complications and worsening of sarcoid chest involvement.

Conclusion

This case series suggests that infliximab might be useful for the treatment of sarcoidosis with sinonasal involvement.     

Atteintes rénales de la sarcoïdose

Sarcoidosis is a chronic multisystemic inflammatory disorder of unknown etiology, characterized by the presence of non-necrotizing epithelioid and giant cell granulomas. Various renal manifestations have been reported in patients with sarcoidosis. Disorders of bone and mineral metabolism related to the overexpression of 25-hydroxyvitamin-D1α-hydroxylase by alveolar and granuloma macrophages are frequently associated with sarcoidosis. Hypercalcemia and hypercalciuria are a major cause of renal injury predisposing to pre renal azotemia, acute tubular necrosis, nephrolithiasis and nephrocalcinosis. Therapeutic management of hypercalcemia includes preventive measures (limited sunlight exposure, limited vitamin D and calcium intakes, and adequate hydration) and specific treatment in cases of severe hypercalcemia (corticosteroid therapy, chloroquine or ketoconazole). Granulomatous tubulointerstitial nephritis is the most common renal lesion associated with sarcoidosis leading to end stage renal disease in some patients. In these cases, interstitial fibrosis seems to appear early in the course of sarcoidosis and is a major prognostic factor requiring rapid corticosteroid therapy to reduce the risk of severe renal impairment. Membranous nephropathy seems to be the most frequent glomerular disease that may occur in association with sarcoidosis. Among kidney allograft recipients, the risk of recurrence of granulomatous tubulointerstitial nephritis is high and may have a negative impact on the graft survival.     

Granulomatoses rénales : étude rétrospective de 44 observations

Purpose

Granulomatous interstitial nephritis (GIN) are identified in 0.5 to 1,3% of all renal biopsies. Renal outcome and treatment modalities are not clearly established in the literature.

Methods

We retrospectively analyzed a case series of 44 GIN identified among all renal biopsies performed between 1984 and 2005 in the Rhône-Alpes area.

Results

The study population included 25 men and 19 women with a mean age of 56 years, and mean diagnostic delay was 11 months. Renal function was severely impaired (mean creatinine clearance 24 mL/min). Proteinuria was observed in 77% (mean value 0,9 g/24 h) of the patients and associated with microscopic hematuria and leukocyturia in 30% and 25%, respectively. The most common diagnosis was sarcoidosis (25%, n = 11), followed by drug-induced GIN (9%, n = 4), tuberculosis (6,8%, n=3), hemopathy-related paraneoplastic GIN (6,8%, n = 3), HIV infection (n = 1) and chronic renal allograft rejection (n = 1). In other patients, no aetiology was found (48%, n = 21). Severity of renal failure justified hemodialysis in 34% (n = 15) of the patients. Three patients underwent renal transplantation. Nonetheless, renal outcome was generally favorable: renal function improved in 41% (n = 18) and stabilized in 34% (n = 15) of patients.

Conclusions

Sarcoidosis, drug-induced and infections represent the main causes of GIN. Histologic features are not specific enough to determine the aetiology. Corticosteroids is the gold standard in sarcoidosis, drug-induced, and idiopathic GIN. Treatment is etiologic in the other cases.