Connective Tissue Diseases

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Are  autoimmune  diseases  of  unknown  etiology  that  affects  the  skin,  blood  vessels  and  internal  organs. 

Clinical presentation  Scleroderma 

  • Localized scleroderma- shiny plaques on the extremities, scalp, hands
  • Systemic scleroderma - skin involvement, Raynaud phenomena, internal organ involvement

Lupus erythematosus 

  • Discoid  Lupus  Erythematosus-  hypo/depigmented  plaques,  follicular  plugging  on  the face mostly
  • Systemic Lupus - malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disease

Dermatomyositis 

  • cutaneous-gottrons papules, heliotrope rash
  • proximal muscle weakness- inability to comb hair
  • inability to rise when squats

Investigations 

  • FBP and ESR
  • LFTS
  • RFTS
  • Urinalysis
  • Autoantibodies -  ANA, anti-dsDNA, anti-centromere, anti-scleroderma 70, Anti-phospholipids, Anti- SM
  • Creatine kinase levels
  • Biopsy
  • Monitoring liver, renal and blood count 3 monthly
  • RBG every 3 months for patients on corticosteroids for long

Non-pharmacology Treatment 

  • Ophthalmology review before starting hydroxychloroquine and every 6 months after.
  • Monitor blood pressure
  • Use of sun protective factor (SPF) lotion and clothing especially for dermatomyositis and Lupus.
  • Keep extremities warm
  • Physiotherapy
  • Involve physician

Pharmacological Treatment  Deworm- Albendazole 400mg stat 

Localized scleroderma: 

D: clobetasol propionate cream / ointment (topical) 0.05% 12hourly 

Systemic scleroderma 

A: prednisolone (PO) 0.5-1mg /kg 24hourly for stabilizing then taper slowly 

AND  

S: methotrexate (PO) 7.5- 20mg weekly 

Discoid Lupus Erythematosus 

D: clobetasol propionate cream/ointment (topical) 0.05% 12hourly 

AND       

S: hydroxychloroquine (PO) 200mg 24hourly for not less 3months 

Systemic Scleroderma 

D: clobetasol propionate cream/ointment (topical) 0.05% 12hourly 

AND 

A:  prednisolone  (PO)  0.5-  1  mg  24hourly  for  4weeks  then  taper  to  lowest  level  that  is effective.  

AND 

S: methotrexate (PO) 7.5- 20mg weekly 

AND 

A:  omeprazole  (PO)20mg  24hourly  when  needed  for  patients  with  gastritis,  peptic  ulcer disease. 

Dermatomyositis 

A: prednisolone (PO) 1mg/ kg 24hourly for 12weeks till improvement then start tapering to lowest effective dose 

AND 

S: methotrexate (PO) 7.5-20mg weekly for at least 6months