EUH Dressler Conference: Review of polymyositis

The Bottom Line: Inflammatory idiopathic myopathies (IIM) are a group of rare autoimmune diseases characterized by proximal skeletal muscle weakness, raised muscle enzymes and extramuscular organ involvement, most frequently the lungs, resulting in interstitial lung disease (ILD). Numerous autoantibodies are associated with the disease, many linked to different clinical phenotypes.  Polymyositis predominantly presents with proximal symmetrical muscle weakness, while dermatomyositis is characterized by skin and muscle involvement; both are associated with extramuscular features.

Clark, K., & Isenberg, D. (n.d.). A review of inflammatory idiopathic myopathy focusing on polymyositis. European Journal of Neurology., 25(1), 13-23.

The main aims of treatment are to suppress inflammation, improve muscle power and prevent chronic damage to muscles and extramuscular organs. However, there is a lack of robust data to guide treatment.  Glucocorticoids remain the mainstay of treatment in IIM. Initial dosing is approximately 0.5 mg/kg of prednisolone, but the many side effects of steroids encourage a reducing regime over the first 2 months.  Methotrexate and azathioprine are often used as first line disease modifying anti-rheumatic drugs. A Cochrane review found insufficient evidence of improved efficacy using one DMARD (methotrexate, azathioprine or cyclosporine) in combination with corticosteroids in preference to another.

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VA Resident Report: What is the distribution of arthritis associated with systemic infection?

The Bottom Line: Staphylococcus aureus is the main cause of septic arthritis involving native joints, although many other organisms are encountered also. In our center, neither the distribution nor the antibiotic susceptibility profiles of the causative organisms changed significantly over the last 30 years.

Table 1 Organisms responsible for septic arthritis.  Page 439

Dubost, J., Couderc, M., Tatar, Z., Tournadre, A., Lopez, J., Mathieu, S., & Soubrier, M. (2014). Three-decade trends in the distribution of organisms causing septic arthritis in native joints: Single-center study of 374 cases. Joint, Bone, Spine, 81(5), 438-440.

In this study, the distribution and antibiotic susceptibility profile of the organisms responsible for septic arthritis showed little change over the 30-year study period. Importantly, no significant increase in MRSA was noted, in keeping with a previous study. These findings do not support the use in our center of broader-spectrum antibiotics in patients for whom empirical antibiotic therapy is deemed necessary.

EUHM Resident Report: Does pregnancy increase or decrease the risk of lupus flares?

The Bottom Line: SLE patients with stable lupus nephritis demonstrates that flares develop in almost 20% of pregnancies, but flares respond to prompt and adequate therapy. On the same level, contraception and an optimal timing for pregnancy should be discussed with patients, particularly when anti-phospholipid and anti-Ro/SSA antibodies are present.

References: Doria, A., Gershwin, M., & Selmi, C. (2016). From old concerns to new advances and personalized medicine in lupus: The end of the tunnel is approaching. Journal of Autoimmunity, 74, 1-5.

Summary: The best news in SLE is related to pregnancy management as the disease most frequently affects women of childbearing age. If in the past pregnancy was “contraindicated” because of the high risk of flares, pregnancies can now be successful after the diagnosis SLE, even if with some precautions .

VA Resident Report: What are the SLE overlap syndromes?

The concept of overlap syndromes implies the occurrence of two or more well-defined connective tissue diseases in the same patient. OSs are not frequent, and their descriptions in the literature are limited to a few case reports and case series.

Iaccarino, L., Gatto, M., Bettio, S., Caso, F., Rampudda, M., Zen, M., . . . Doria, A. (n.d.). Overlap connective tissue disease syndromes. Autoimmunity Reviews., 12(3), 363-373.

Overlap Syndromes have been defined as entities satisfying classification criteria of at least two connective tissue diseases occurring at the same or at different times in the same patient. Connective tissue diseases include systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, polymyositis/dermatomyositis, and Sjögren syndrome. Every combination between these disorders has been reported. In some overlap syndromes a specific autoantibody has been identified, supporting the hypothesis that these syndromes are not a mere association of two or more connective tissue diseases in the same patient, but a well-defined clinical entity with specific clinical characteristics

What is the sensitivity and specificity of Yamaguchi Criteria for the diagnosis of Adult Onset Stills Disease?

Lian, Fan, et al. “Clinical features and hyperferritinemia diagnostic cutoff points for AOSD based on ROC curve: a Chinese experience.” Rheumatology international 32.1 (2012):189-92.

Statistical ability of combined Yamaguchi criteria and ferritin for AOSD diagnosis

Yamaguchi criteria for AOSD had a sensitivity of 96.2% and a specificity of 92.1%.

Table 3 shows the sensitivity and specificity of combined Yamaguchi criteria and hyperferritinemia.

Table 3  Combined Yamaguchi criteria and hyperferritinemia

Sensitivity (%)                   Specificity (%)

Ferritin ≥750 µg/L                   83.5                                98.8

Ferritin ≥1,250 µg/L                 70.9                               99.3

Ferritin ≥2,500 µg/L                 43.0                                99.9

Causes and significance of markedly elevated serum ferritin levels

Bottom line: Out of all adult patients with a least 1 serum ferritin level >1,000μg/L from 2008-2010 (n=627), 153 had a malignancy, and 136 had iron-overload syndromes. Average ferritin level for the 6 patients with either adult-onset Still’s disease, systemic juvenile idiopathic arthritis, or hemophagocytic lyphohistiocytosis/macrophage activation syndrome was 14,242μg/L.
Source: Moore, Charles, MichelleOrmseth, and HowardFuchs. “Causes and significance of markedly elevated serum ferritin levels in an academic medical center.” Journal of clinical rheumatology 19.6 (2013):324-8.

 

What is the epidemiology of clinically significant pulmonary hypertension in patients with sarcoidosis?

Bottom line:  Pulmonary hypertension can affect patients at any stage of sarcoid disease, but is most prevalent at more advanced stages.  Patients with sarcodosis who present with dyspnea on exertion, cough, chest pain or palpitations could have pulmonary hypertension, which is associated with a poorer prognosis, but these symptoms can also be associated with the sarcoidosis alone or other conditions.

Summary: Cordova FC, et al. Sarcoidosis-associated pulmonary hypertension. Curr Opin Pulm Med. 2013 Sep;19(5):531-7. doi: 10.1097/MCP.0b013e328363f4a3.

Shino MY, et al. Sarcoidosis-associated pulmonary hypertension and lung transplantation for sarcoidosis. Semin Respir Crit Care Med. 2014 Jun;35(3):362-71. doi: 10.1055/s-0034-1376863.
According to these recent reviews, PH occurs in 5-79% of patients with higher rates among patients with “advanced fribrocystic disease.”  The incidence of PH also varies according to the definition of PH.

In looking at clinical presentation of patients with sarcoidosis and PH, one study of 106 patients found no difference between sarcoidosis patients with and without PH in frequency of presentation with dyspnea on exertion, cough, palpitations, or chest pain. Radiographic findings were not significantly different.  (Chest. 2005 Sep;128(3):1483-9. Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension. Sulica R, et al.)

Both Cordova and Shinu reference studies finding a statistically significant difference in the six minute walk test between patients with and without PH.

Pulmonary hypertension is associated with a poorer prognosis.  Shino and colleagues cite a retrospective study of 404 patients with sarcoidosis awaiting lung transplant in the US.   Three factors for increased risk of mortality included presence of PH, the amount of supplemental oxygen needed, and African American race.  (See Prognosis of Pulmonary Hypertension section.)

Sensitivity and specificity of magnetic resonance imaging (MRI) for diagnosis of osteomyelitis

Bottom line: Sensitivity for diagnosing two types of osteomyelitis, chronic and foot, varied from 77-100%; likewise, specificity varied from 40-60%.

Termaat, M F, et al. “The accuracy of diagnostic imaging for the assessment of chronic osteomyelitis: a systematic review and meta-analysis.” JBJS The Journal of Bone & Joint Surgery 87.11 (2005):2464-2471.
Systematic review included 5 MRI studies. Pooled sensitivity for MRI in these five studies was 84% (95% confidence interval, 69% to 92%), and the pooled specificity was 60% (95% confidence interval, 38% to 78%).

Kapoor, Alok, et al. “Magnetic resonance imaging for diagnosing foot osteomyelitis: a meta-analysis.” Archives of internal medicine 167.2 (2007):125-132.
In this systematic review of 16 studies, the diagnostic odds ratio (DOR) was 42.1 (95% confidence interval [CI], 14.8-119.9), sensitivity was 77%-100%, and specificity was 40%-100%. MRI’s specificity was 82.5% at the clinically relevant cut-point of 90% sensitivity.