God is our Guide  Number 1 site for helping reverse diseases on Planet Earth

 

 

 
     Home
      Diagnosis
      Treatment
      Pathology
      Variants
      CIDP info
      Fibromyalgia
     IVIG
     Diet anti-inflammatory
     Burning  Feet Home
     Services Page
     Chronic Fatigue
     Autoimmune diseases
     Prognosis
     Bible healing
      Celiac disease
Autoimmune self attack

CMT

Cholesterol drugs & Bleeding

What is autoimmune

Toxic Baby products

Infants  and women omega-3

 Selenium

Basil

Bay leaves

Eliminate insulin implants

Sugar treatment

Heart FAILURE

Irregular periods

Myasthenia diet

Prophets

cancer survivor

Tomato as a medicine

New Vaccine

Risk of heart disease & stroke 

Depression and breast cancer

Kidney stone removal 

Alopecia general

Personality

Skin hair nail spa

Anemia and celiac disease

Lower cholestrol

 Inflammatory muscle Disorders-2

  Complete  guide on alternatives treatment of autoimmune disease please read our e-book 

 

 

 

Presentation and management of idiopathic inflammatory muscle disease: four case reports  from a series of 78 patients -2

Continued from page -1

Severe myositis, as evidenced by the CK, does not necessarily imply diffuse myositis, as demonstrated in this case by the MRI. Indeed a patchy distribution on MRI is characteristic of these diseases, and explains why a normal muscle biopsy is not incompatible with severe disease. The lack of inflammatory cells in the muscle biopsy in this case may also be due to the vasculitic basis of dermatomyositis, leading to ischaemic muscle necrosis [13]. In other situations a normal biopsy may result from steroid treatment. However, in this case, central nucleation is a feature of regenerating muscle fibres, and therefore demonstrates an active pathological state.

In our series of 78 patients, 23 (29%) had pharyngeal or oesophageal involvement. Intravenous Ig was used to treat dysphagia in the four most severe cases, with complete recovery occurring in all, including the case described. Cyclosporin was used in this case rather than azathioprine as it is available in liquid form and is therefore more convenient for long‐term use in patients with a PEG tube. This case illustrates the point that patients may need two or more courses of i.v. Ig before they regain normal swallowing. The use of i.v. Ig is discussed in detail in the Discussion.

 

Case 2

Diagnosis: Dermatomyositis, calcinosis, cerebellar vasculitis.

Treatment: Prednisolone, azathioprine, i.v. Ig, diltiazem.

A 51‐yr‐old African woman presented with a 3‐month history of facial swelling, tenderness and weakness of the upper arms and thighs, and difficulty swallowing. On examination there was periorbital oedema and considerable proximal muscle weakness. The serum total CK was 8585 U/l, ESR 35 mm/h and no autoantibodies including ANA and Jo‐1 were present. An EMG showed patchy low‐amplitude polyphasic potentials and a full interference pattern in keeping with a myopathic process. Muscle biopsy from the thigh was normal. Barium swallow and echocardiogram were normal. The features were characteristic of dermatomyositis.

Following initial treatment with prednisolone and azathioprine the weakness slowly improved and the serum CK fell to 98 U/l. Prednisolone was successfully tapered to a low dose and she remained well. Three years later she developed calcinosis in the tissues of her buttocks and upper arms and a skin biopsy revealed vasculitis (Fig. 1). A year later she relapsed, became weak, the ESR rose to 58 mm/h and the CK remained normal. An MRI of both thighs showed patchy increased signal on inversion recovery images, and muscle biopsy from the thigh revealed basophilic fibres with lymphocytic infiltration. At the same time she developed ataxia and dizziness, and an MRI of the brain revealed a left cerebellar hemisphere infarct (Fig. 2), which was presumed to be due to vasculitis. She received two courses of i.v. Ig (2 g/kg) resulting in an improvement in muscle weakness; and dizziness resolved spontaneously. The calcinosis was treated with diltiazem, up to 360 mg daily, bu>Comment

Calcinosis is a feature of dermatomyositis that is more frequently recognized in children. We have one other adult case of dermatomyositis with calcinosis in our series, in which, in contrast to this report, the calcinosis improved considerably following treatment with the calcium channel blocker diltiazem.

The primary pathological process in dermatomyositis is vasculitis within skeletal muscle , and is demonstrated in this case in the skin biopsy. Systemic vasculitis is also reported, but involvement of the central nervous system (CNS) is rare and more classically described in children . In this case the appearances on the MRI were of ischaemia, which, in the absence of risk factors for thromboembolic disease, we feel was most likely to be due to vasculitis. We have seven other cases of systemic vasculitis in our series (9%), and one other case of CNS vasculitis, all in patients with dermatomyositis.

 

Fig. 1.
View larger version:
Fig. 1.

Case 2. Haematoxylin and eosin section of skin showing a florid vasculitis with fibrinoid necrosis (arrow). In addition to the perivascular inflammatory infiltrate there is a diffuse acute inflammatory infiltrate throughout the dermis.

 
Fig. 2.
View larger version:
Fig. 2.

Case 2. T2‐weighted MR image of brain showing high signal in the left cerebellar hemisphere in keeping with an area of ischaemia (arrow).

Please continue to page-3