Inflammatory conidtions - part 2

Question 1

What is sarcodosis?

Answer 1

Chronic Glanulomatous disorder of unknown aeitology
(granulomas - in sarcodosis are non - caseating - not associated with necrotic changes)

Granulomas form in lungs & other organs ————————– > caused by accumulation of lymphocytes & macrophage.

Diagnosed by excluding other granulomatous lung diseases e.g tuberculosis , histoplasmosis etc.

SIGNS & SYMPTOMS

• Cough
• Dyspnoea
• wheezing
• rhonchi (continous gurgling or bubbling sounds on inhalation & exhalation)
• Chronic fatigue
• arthralagia
• lymphadenopathy (particularly cervical & submandibular)
• Photophobia , painfull red eye , blurred vision
  (symptoms of uveitis)
• arthalgia.

UNCOMMON

Erythema nodosum

RISK FACTORS

• Age - 20 to 40 (most common)
• FHx of Sarcodosis
• Scandinavian origin.

Chronic Glanulomatous disorder of unknown aeitology

Granulomas form in lungs & other organs ————————– > caused by accumulation of lymphocytes & macrophage.

Diagnosed by excluding other granulomatous lung diseases e.g tuberculosis , histoplasmosis etc.

SIGNS & SYMPTOMS

• Cough
• Dyspnoea
• wheezing
• rhonchi (continous gurgling or bubbling sounds on inhalation & exhalation)
• Chronic fatigue
• arthralagia
• lymphadenopathy (particularly cervical & submandibular)
• Photophobia , painfull red eye , blurred vision
  (symptoms of uveitis)
• arthalgia.

UNCOMMON

Erythema nodosum

RISK FACTORS

• Age - 20 to 40 (most common)
• FHx of Sarcodosis
• Scandinavian origin.

caseting granulomas cause by infection by foreign substance e.g TB.

Question 2

Diagnosis of Sarcodosis ?

Answer 2

0 CXR
Bilateral hilar adenopathy ( enlargement of lymph nodesat hilium - along bronchi)

or extensive fibrosis with distortion
(different stages)

0 PFTs

• Serum Urea
• Creatine
• liver enzymes
  ( if liver involved)

0 ECG - to exclude / confirm, cardiac involvement.

PPD of tuberculin ( used to screen tuberclosis - negative (Mantoux TB skin test)

0 FBC - anaemia

0 Serum Calcium - activated marcophages & granulomas - cause dysregulation of production of calcitrol.

NOTE - absensce of beryllium
(Berylliosis - form of metal posioning occuring by inhalation of B / implantation into skin.
Occupational exposure - common cause e.g. aerospace , nuclear industries
(has similar symptoms / signs to sacrodosis).

Consider:

Serum ACE - produce by recently activated macrophages & granulomas. - elevated.

Question 3

Treatment of Sarcodosis ?

If lungs affected

Answer 3

ACUTE RESPIRATORY FAILURE

1ST LINE

Oral prednisolone

if cannot tolerate oral :

IV Corticosteriod
(methylprednisolone sodium succinate) --------> as the improve switched to  oral. 

PLUS ; Ventilatory support & oxygen

PULMONARY DISEASE - ONGOING

stage 1 - no progressive adenopathy

1ST LINE - observation

Symptomatic stage - progressive adenopathy

1ST LINE

oral or inhaled corticosteriod
(adjuncts - Cytotoxics e.g methotrexate , azathropine , hydroxychloroquine - can be used as steriod sparing agents)

2ND LINE - Cytoxics
(adjuncts - oxygen)

end stage lung disease

1ST LINE - lung transplant

Question 4

Treatment of Sarcodosis ?

Cutaneous disease.

Answer 4

erythematous scaly rash

1ST LINE - topical corticosteriods
(adjunct - intra-lesional)

2ND LINE - oral C

3RD LINE - Hydrochloroquine

LUPUS PERNIO WITH SCARRING

1ST LINE - oral C

2ND LINE - Cytotoxics

Lupus pernio isone of the more common forms of cutaneous sarcoidosis. Sarcoidosis is a multisystem disorder characterised histologically by granulomas (a specific type of inflammation). Lupus pernio presents as bluish-red or violaceous nodules and plaques over the nose, cheeks and ears.

Question 5

Treatment of Sarcodosis ?

CNS , Peripheral involvement , cardiac involvement

Answer 5

1ST LINE

Oral pred

2ND LINE

Cytotoxics - metho , azathropine

secondary option - ciclosporin