Synovial chondromatosis

Disease class: Joint diseases

Primary hypertrophic osteoarthropathy

Disease class: Joint diseases

Ainhum

Disease class: Bone resorption

Also known as

• Dactylolysis spontanea
  

This is a disease characterised by spontaneous auto-amputation of the fifth digit of the foot bilaterally. The process begins with the appearance of a groove, on the plantar, medial aspect of the proximal fifth digit. This groove progressively deepens and surrounds the digit until complete amputation occurs.

 

Etymology

• From the Yoruba word ayùn, (to saw, to file).

 

Aetiology

• Unknown.
• Vascular abnormalities may predispose to insufficient circulation, and an increased risk of ainhum.
• Absence of the plantar arch is associated with an increased risk of ainhum
• Walking barefoot is associated with an increased risk of ainhum.

 

Epidemiology

• Predominately seen in African populations. Historically described along the Gold Coast (Gulf of Guinea).

 

Pathophysiology

Grade	Pathological progress
I	groove
II	floor of the groove is ulcerated
III	bone involvement
IV	autoamputation has occurred

 

Management

Grade I-II lesions

• Injection of corticosteroids.
• Excision of the groove, followed by z-plasty can reduce pain and probability of amputation.

Grade III lesions

• Disarticulation of the metatarsophalangeal joint.

Cerebellar diseases

Disease class: Brain diseases

Types

• Cerebellar ataxia
• Cerebellar neoplasms
• Dandy-Walker syndrome
• Miller Fisher syndrome
• Paraneoplastic cerebellar degeneration
• Spinocerebellar degenerations

This refers to diseases caused by pathology of the cerebellum. 

Intracranial hypotension

Disease class: Brain diseases

Idiopathic hypersomnolence

Disease class: Disorders of excessive somnolence

Hyperhidrosis

Disease class: Sweat gland diseases

This term describes excessive sweat production.
The skin of the hands and feet may be excessively moist.

Management

• Antiperspirants: Aluminium chloride

Penile induration

Disease class: Connective tissue diseases

Disease class: Penile diseases

 

Also known as

• Peyronie disease 

 

Definition

• This refers to a progressive thickening of the tissues along the penile shaft, causing contraction and curvature towards the affected area. There is often pain associated with the lesion.

 

Management

Conservative

Minimise any sources of penile trauma

 

Surgical

Aims to correct deformity and restore function.
May cause reduction in penile length or cause chronic pain.

 

Medical 

Injections
Vibration therapy

Cryptogenic organising pneumonia

Disease class: Bronchiolitis Obliterans

Disease class: Idiopathic interstitial pneumonias

A form of bronchiolitis which may develop as a complication of rheumatoid arthritis or amiodarone therapy.

Restless legs syndrome

Disease class: Nervous system diseases
Disease class: Parasomnias

Also known as

• Willis–Ekbom disease (WED)
• Wittmaack–Ekbom syndrome

 

Epidemiology

• Frequency: 2.5–15% of the population

Idiopathic intracranial hypertension

Essential tremor

Essential hypertension

MeSH: Hypertension
ICD-11: Hypertensive diseases 

Essential hypertension refers to hypertension which is not caused by any known diseases (e.g. Glomerulonephritis).
This is one of the most common diseases in elderly patients.

 

Pathophysiology

The underlying mechanism behind this disease is unknown, but it becomes more prevalent with advanced age. It appears to reflect a gradual decline in the body's ability to self-regulate the blood pressure at an appropriate 'setting'.

 

Complications

Acute

• Cerebrovascular accident
• Hypertensive emergency

Chronic

• Hypertensive heart disease
• Hypertensive kidney disease
• Hypertensive retinopathy

Idiopathic pulmonary fibrosis

Disease class: Idiopathic interstitial pneumonias

Idiopathic pulmonary fibrosis (IPF, previously termed cryptogenic fibrosing alveolitis) is a chronic lung condition characterised by progressive fibrosis of the interstitium of the lungs. Whilst there are many causes of lung fibrosis (e.g. medications, connective tissue disease, asbestos) the term IPF is reserved when no underlying cause exists.

IPF is typically seen in patients aged 50-70 years and is twice as common in men.

 

Features

• progressive exertional dyspnoea
• bibasal crackles on auscultation
• dry cough
• clubbing

Diagnosis

• spirometry: classically a restrictive picture (FEV1 normal/decreased, FVC decreased, FEV1/FVC increased)
• impaired gas exchange: reduced transfer factor (TLCO)
• imaging: bilateral interstitial shadowing (typically small, irregular, peripheral opacities - 'ground-glass' - later progressing to 'honeycombing') may be seen on a chest x-ray but high-resolution CT scanning is the investigation of choice and required to make a diagnosis of IPF
• ANA positive in 30%, rheumatoid factor positive in 10% but this does not necessarily mean that the fibrosis is secondary to a connective tissue disease. Titres are usually low

Management

• pulmonary rehabilitation
• very few medications have been shown to give any benefit in IPF. There is some evidence that pirfenidone (an antifibrotic agent) may be useful in selected patients (see NICE guidelines)
• many patients will require supplementary oxygen and eventually a lung transplant

Prognosis

• poor, average life expectancy is around 3-4 years