Cardiac sarcoidosis and coronary artery disease: a two-hit mechanism to left ventricular dysfunction (or is it)?

This report describes a case of cardiac sarcoidosis in a 40-year-old man with minimal risk factors for coronary artery disease who was found to have a critical coronary lesion on angiography performed for declining left ventricular function. The case highlights the diagnostic and therapeutic issues surrounding cardiac sarcoidosis and raises the question of a possible link between sarcoidosis and premature coronary artery disease. It also stresses the importance of ruling out ischemia in any patient with declining left ventricular function.

Calcium and vitamin D metabolism in sarcoidosis.

BACKGROUND: Sarcoidosis associated hypercalcemia (SAHC) may be secondary to excessive levels of 1,25-(OH)2 vitamin D3 produced by autonomous 1-alpha-hydroxylase activity within the granulomas.  The frequency, treatment, and consequences of hypercalcemia remain unclear. STUDY DESIGN AND METHODS: Two patient cohorts were studied.  In Cohort 1, the prevalence of hypercalcemia in 1606 sarcoidosis patients seen during a six year period was analyzed along with treatment and outcome. Cohort 2 consisted of  261 sarcoidosis patients with measured 25-(OH) vitamin D3 and 1,25-(OH) vitamin D3 levels. In forty patients, serial levels of 25-(OH) vitamin D3 and 1,25-(OH) vitamin D3 were measured at least three months apart without change in therapy. RESULTS: SAHC was identified in 97 of 1606 (6%) of patients studied and additional nine (0.6%) patients had primary hyperparathyroidism. Post treatment follow up was available in 86 SAHC patients. Hypercalcemia improved in >90% of patients, including eight patients treated solely with vitamin D supplement withdrawal. Renal insufficiency, documented in 41 (42%) of SAHC patients, improved with hypercalcemia treatment.  In 80% of Cohort 2 patients low 25-(OH) vitamin D3 levels were measured with only one patient having a low 1,25(OH)2 vitamin D3 level. Elevated 1,25(OH)2 vitamin D3 levels, which were measured in 11% of patients, were higher for those with a history of hypercalcemia. CONCLUSION: Sarcoidosis associated hypercalcemia, which is often accompanied by renal insufficiency, responds to treatment of sarcoidosis and withdrawal of vitamin D supplementation. Measurement of serum vitamin 1,25(OH)2 vitamin D3 appears to best evaluate vitamin D status in sarcoidosis patients.

C-reactive protein predicts response to infliximab in patients with chronic sarcoidosis.

BACKGROUND: This study assessed the value of C-reactive protein as a predictor of disease severity and response to infliximab therapy in patients with chronic pulmonary sarcoidosis. DESIGN: Sera were collected through week 52 from 138 patients with chronic pulmonary sarcoidosis who received placebo or infliximab in a randomized, double-blind, placebo-controlled study. We evaluated the response to therapy by baseline CRP using a dichotomous cutpoint (0.8 mg/dL) for the change from baseline in percent-predicted forced vital capacity (FVC), Saint George's Respiratory Questionnaire (SGRQ), 6-minute walk distance (6MWD), Borg's CR10 dyspnea score, and Physician Organ Assessment (POA). RESULTS: CRP was elevated in 36% of patients at baseline, and was significantly reduced by infliximab by week 2. Among patients with elevated baseline CRP, infliximab-treated patients improved significantly compared with placebo-treated patients in percent-predicted FVC (+2.5 versus -2.6%), 6MWD (+8.0 versus -34.1), Borg's CR10 dyspnea score (pre-6MWD -0.8 versus +0.9, post-6MWD -1.1 versus +0.8), and POA (-3.1 versus -0.3). Patients with lower CRP levels at baseline did not show significant differences between the placebo and infliximab groups in most endpoints evaluated. CONCLUSIONS: In chronic sarcoidosis patients, elevated CRP appears to identify a subset with more severe disease who may respond better to treatment with infliximab.

Minor salivary gland inflammation in Devic's disease and longitudinally extensive myelitis.

Devic's disease is often considered as a variant of multiple sclerosis (MS). However, evidence suggests that Devic's disease may be distinct from MS. Devic's disease can coexist with connective tissue diseases, particularly Sjögren's disease, but this association is rare with MS. Diagnosis of Sjögren's disease in patients with neurological symptoms is often difficult. During early stages of Sjögren's disease, patients may not fulfill all criteria for Sjögren's disease. A high percentage of patients with Sjögren's disease have inflammatory infiltrates in minor salivary glands, and this may be a reliable indicator of early or subclinical disease. We show high prevalence (80%) of salivary gland inflammation in Devic's disease and longitudinally extensive transverse myelitis (LETM). We diagnosed 16 patients with Devic's disease, and 2 of these satisfied criteria for Sjögren's disease as did 2 of 9 patients with LETM. Anti-SSA/B titers were infrequently elevated. Although most did not satisfy criteria for Sjögren's disease. 9 of 12 Devic's disease patients and 7 of 8 LETM patients had severe salivary gland inflammation. Thus: (1) patients with Devic's disease or with LETM who have positive labial biopsies but do not satisfy criteria for Sjögren's disease could have subclinical Sjögren's diseases. Alternatively, (2) as patients with Devic's disease have elevated titers of several autoantibodies, so there may exist a set of antibodies that react with antigens in minor salivary glands and cause inflammation. Minor salivary gland biopsy is more sensitive than anti-SSA/B serology in providing histological evidence for possible Sjögren's disease with CNS lesions.