Systemic complaints (fever, malaise, fatigue, weight loss, myalgias)
Diffuse Systemic<unk> erosis
hypo- or hyper- pigmentation of skin, extending from fingers and toes proximally, also involving chest and abdominal wall
Pigment is perserved around hair follicles leading to salt and pepper appearance  Hypopigmentation in Diffuse<unk> , courtesy of Regional Derm website
Symmetric hand edema and Raynaud's phenomenon
Abrupt disease presentation; worse in first 18 months, then either improvement or worsening to involve internal organs
CREST Syndrome
Longstanding Raynaud's
Skin thickening and fibrosis distal to elbows and knees, and on face
Subcutaneous calcinosis
Esophageal dysmotility<unk> erodactyly
Telangiectasia
Indolent course
hypo- or hyper- pigmentation of skin, extending from fingers and toes proximally, also involving chest and abdominal wall
Pigment is perserved around hair follicles leading to salt and pepper appearance  Hypopigmentation in Diffuse<unk> , courtesy of Regional Derm website
Symmetric hand edema and Raynaud's phenomenon
Abrupt disease presentation; worse in first 18 months, then either improvement or worsening to involve internal organs
Longstanding Raynaud's
Skin thickening and fibrosis distal to elbows and knees, and on face
Subcutaneous calcinosis
Esophageal dysmotility<unk> erodactyly
Telangiectasia
Indolent course
Renal crisis
Causes are vessel narrowing and subsequent ischemic kidney disease
Leads to hypertensive emergency, acute renal failure, and microangiopathic hemolytic anemia
Occurs during first 5 years of the disease
Was the most common cause of death prior to ACEi usage
Severe renal crisis is life threatening with incidence of 8-10% in Limited and 10-20% in diffuse
Similar to TTP/HUS with microangiography
Poor prognosis with sudden onset of hypertension, encephalopathy, CVA, retinopathy
Risk factors are rapidly progressing diffuse<unk> , high dose glucocorticoid use, cyclosporine therapy, presence of anti-RNA-polymerase antibodies.
Pulmonary
Respiratory failure
Interstitial lung disease, leading to pulmonary fibrosis
Found in dorsal portion of both lower lobes, but can extend to upper lobes in severe diease
Poor prognosis with severe pulmonary impairment.  42% die within 10 years of disease onset
ARDS
Aspiration pneumonitis
Pulmonary hypertension
Gradually progressive exercise breathlessness
Fatigue
Hoarseness from nerve palsy caused by dilated pulmonary artery stem
Alveolar hemorrhage
Hemoptysis, infiltrates on CXR, anemia.
Cardiac
Acute heart failure
Can be from diastolic dysfunction, malignant hypertension during renal crisis, and decompensated pulmonary hypertension
Cardiopulmonary complications are the most common causes of<unk> -related death, with cardiac causes and pulmonary fibrosis being the most common.
Anginal pain from right ventricle ischemia and low cardiac output
Causes are vessel narrowing and subsequent ischemic kidney disease
Leads to hypertensive emergency, acute renal failure, and microangiopathic hemolytic anemia
Occurs during first 5 years of the disease
Was the most common cause of death prior to ACEi usage
Severe renal crisis is life threatening with incidence of 8-10% in Limited and 10-20% in diffuse
Similar to TTP/HUS with microangiography
Poor prognosis with sudden onset of hypertension, encephalopathy, CVA, retinopathy
Risk factors are rapidly progressing diffuse<unk> , high dose glucocorticoid use, cyclosporine therapy, presence of anti-RNA-polymerase antibodies.
Was the most common cause of death prior to ACEi usage
Similar to TTP/HUS with microangiography
Poor prognosis with sudden onset of hypertension, encephalopathy, CVA, retinopathy
Risk factors are rapidly progressing diffuse<unk> , high dose glucocorticoid use, cyclosporine therapy, presence of anti-RNA-polymerase antibodies.
Respiratory failure
Interstitial lung disease, leading to pulmonary fibrosis
Found in dorsal portion of both lower lobes, but can extend to upper lobes in severe diease
Poor prognosis with severe pulmonary impairment.  42% die within 10 years of disease onset
ARDS
Aspiration pneumonitis
Pulmonary hypertension
Gradually progressive exercise breathlessness
Fatigue
Hoarseness from nerve palsy caused by dilated pulmonary artery stem
Alveolar hemorrhage
Hemoptysis, infiltrates on CXR, anemia.
Found in dorsal portion of both lower lobes, but can extend to upper lobes in severe diease
Poor prognosis with severe pulmonary impairment.  42% die within 10 years of disease onset
Gradually progressive exercise breathlessness
Fatigue
Hoarseness from nerve palsy caused by dilated pulmonary artery stem
Hemoptysis, infiltrates on CXR, anemia.
Acute heart failure
Can be from diastolic dysfunction, malignant hypertension during renal crisis, and decompensated pulmonary hypertension
Cardiopulmonary complications are the most common causes of<unk> -related death, with cardiac causes and pulmonary fibrosis being the most common.
Anginal pain from right ventricle ischemia and low cardiac output
Can be from diastolic dysfunction, malignant hypertension during renal crisis, and decompensated pulmonary hypertension
Scleroderma
Systemic Lupus Erythematosus
Dermatomyositis
Sjögren syndrome
Vitiligo
Acute arterial ischemia
Brash syndrome
Atheroembolism 