Riedel's thyroiditis association with IgG4-related disease.

CONTEXT: IgG4-positive (+) plasma cells have been reported in both Riedel's thyroiditis (RT) and Hashimoto's thyroiditis (HT). These cells are the hallmark of IgG4-related disease (IgG4-RD). OBJECTIVE: We sought to determine whether RT is part of IgG4-RD spectrum. DESIGN, SETTING AND PATIENTS: This was a case-control study performed at a tertiary medical centre. We included RT cases from the period 1958 to 2008 that had sufficient paraffin-embedded tissue for IgG4 immunostaining. Controls were patients with HT, age and gender matched, with similar pathology criteria. MAIN OUTCOME MEASURE: The main outcome measures were the intensity of the IgG4 staining and the clinical and histological correlates with IgG4-RD. RESULTS: Six pairs of RT and HT were analysed. The mean age was 44·7 years. In both groups, 5/6 cases had positive IgG4 staining. The mean number of IgG4 + cells/ HPF, normalized to the degree of inflammation, was 3·2 ± 3·0 SD (RT) vs 0·9 ± 0·7 (HT), P = 0·15, for fibrotic areas and 2·1 ± 2·3 SD vs 1·0 ± 0·8 (P = 0·39) for areas with lymphoid aggregates. We found the number of IgG4 +  cells in RT to be inversely correlated with the duration of disease (P = 0·046). Three RT cases had associated comorbidities from the IgG4-RD spectrum while none of the HT cases had such conditions. CONCLUSIONS: Riedel's thyroiditis is a component of IgG4-RD with the density of the IgG4 +  lymphocytic infiltrate being time dependent. In this small study, we did not identify differences in IgG4 infiltration between RT and HT, minimizing the utility of this marker in RT diagnosis.

A rare case of ectopic retrosternal goiter.

It is important for the clinician to be familiar with interpreting a variety of radiological modalities that provide vital information that will aid in the preoperative planning, counseling, and subsequent management of patients with retrosternal goiter.

FLAIL MITRAL VALVE: A RARE COMPLICATION OF A THYROID STORM.

OBJECTIVE: Thyroid storm is a life-threatening presentation, with heart failure and tachyarrhythmias being common manifestations. This case highlights that a flail mitral valve from chordae tendineae rupture can be a cause of worsening heart failure and cardiogenic shock in a thyroid storm, albeit a rare complication. METHODS: We describe a patient who was admitted for a thyroid storm precipitated by pneumonia, who later developed an acute flail mitral valve from chordae tendineae rupture. RESULTS: A 55-year-old woman with no past medical history was admitted with fever, dyspnea, lower limb swelling, and hemoptysis. She was febrile, tachycardic, and in fluid overload. Her heart sounds were dual, and no murmurs were heard. Initial investigations indicated primary hyperthyroidism and pneumonia. She was diagnosed with a thyroid storm precipitated by pneumonia, complicated by heart failure. Her Burch-Wartofsky score was 70. She was started on intravenous hydrocortisone, oral propylthiouracil, oral Lugol's iodine, and oral cholestyramine, together with intravenous amoxicillin-clavulanate and intravenous furosemide. She continued to deteriorate in the medical intensive care unit, with worsening hypoxia and hypotension. Echocardiography showed an acute flail posterior mitral valve leaflet with torrential mitral regurgitation from rupture of the chordae tendineae. She subsequently underwent a bioprosthetic mitral valve replacement. CONCLUSION: An acute flail mitral valve precipitated by thyroid storm leading to refractory cardiogenic shock is rare. Factors contributing to the rupture of valve chordae tendineae include the effect of hyperthyroidism on papillary muscle function, a hyperdynamic circulation leading to vulvular stress, as well as pre-existing mitral valve pathology.

Clinical Experience with Rituximab and Intravenous Immunoglobulin for Pretibial Myxedema: A Case Series.

Background: Severe pretibial myxedema (PTM) can be difficult to manage, highlighting the need to investigate newer therapies. Rituximab (RTX) and intravenous immunoglobulin (IVIg) have been tried in Graves' orbitopathy. Since PTM and orbitopathy share a similar underlying pathophysiology, this study aimed to explore these therapies for progressive PTM. Methods: The electronic database was screened for PTM patients evaluated at the Mayo Clinic, Rochester, from 2002 to 2016, and three patients who received IVIg and five who received RTX are reported. PTM pattern was classified as non-pitting edema, plaque and induration, nodular/nummular, and elephantiasis. PTM was confirmed by biopsy in six patients. Results: The patients' median age was 53.8 years, 75% were female, and all but one patient were either active or former smokers. All patients were euthyroid and had progressed despite various therapies prior to starting these agents. Six patients had a plaque and induration pattern, and two had a nodular pattern with elephantiasis. After therapy, six (75%) patients had PTM stability or improvement both subjectively and objectively (80% with RTX and 66% with IVIg). The three patients (one in the IVIg group and two in the RTX group) who had subjective improvement had a plaque pattern. One patient with elephantiasis had a transient response to IVIg and another had stability after RTX. Thyrotropin receptor antibody values and orbitopathy also improved in patients who demonstrated PTM improvement. No serious adverse events were reported, but one patient each had transient hypertension and injection-site thrombophlebitis after IVIg. Conclusions: Immunomodulation therapy was followed by PTM improvement or stability in most patients, with a slightly better response after RTX compared to IVIg. A validated response assessment instrument and larger series of patients are required to determine if the underlying disease process could be curtailed with these agents.

Vertebral compression fractures associated with yoga: a case series.

BACKGROUND: The importance of exercise in skeletal health is increasingly recognized by both patients and providers. However, the safety of prescribed or recreational exercise in at-risk populations remains under-reported and under-publicized. Yoga has gained widespread popularity due to its physical and psychological benefits. When practiced in a population at increased fracture risk, however, some yoga poses may increase fracture risk, particularly at the spine, rather than increasing BMD as noted in recent popular press reports. CASE REPORT: Nine subjects (8 women) with a median age of 66 years (range 53-87), developed vertebral compression fracture (VCF) one month to six years after initiating yoga-associated spinal flexion exercises (SFE). VCF presented with back pain and occurred in the thoracicspine (N.=6), lumbar-spine (N.=4) and cervical-spine (N.=1). Four patients had osteoporosis by BMD criteria prior to VCF and 2 had osteopenia (median T-score -2.35; range -3.3 to +2.0). Interestingly, all patients had their lowest T-scores at the spine. Three patients had a history of fragility fracture prior to the index VCF. While one patient had primary hyperparathyroidism and another was treated with high dose prednisone, no other risk factors for bone loss including medications or secondary osteoporosis causes were identified in the other patients. CLINICAL REHABILITATION IMPACT: This study identified patients in whom increased torsional and compressive mechanical loading pressures occurring during yoga SFE resulted in de novo VCF. Despite the need for selectivity in yoga poses in populations at increased fracture risk, both scientific and media reports continue to advertise yoga as a bone protective activity. Accordingly, yoga is misconceived as a 'onesize-fits-all' prescription. Instead, the appropriate selection of patients likely to benefit from yoga must be a cornerstone of fracture prevention.

Thyroid dysfunction in adult hematopoietic cell transplant survivors: risks and outcomes.

The incidence and risk factors for thyroid disorders among adult survivors of allogeneic hematopoietic stem cell transplantation (alloHCT) is not well known. This study evaluated incidence and risk factors of thyroid dysfunction in 259 adult alloHCT recipients who had survived for 2 years or more after transplant. Median follow-up was 4.6 years (range; 2.3 to 15.4 years). The 5-year cumulative incidence of thyroid dysfunction was 30.5% (79 of 259 patients). Majority of thyroid abnormalities were observed beyond 2 years after alloHCT. Subclinical hypothyroidism was the most common thyroid abnormalities accounting for 89% of cases (71 patients). Among these, treatment with thyroid replacement was eventually started in 44% (31 patients). Based on the multivariate analysis, thyroid abnormality was more likely in patients who received high-dose (≥12 Gy) total body irradiation (TBI)-based conditioning regimen compare to low-dose TBI-based (≤500 cGy) and non-TBI-conditioning regimen (p = 0.032). This risk was further augmented directly proportional with the time from alloHCT. These data identify high-dose TBI-conditioning regimen as a risk factor for thyroid dysfunction in adult recipients of alloHCT.