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Pulmonary Pathology Online

Pathology of Sarcoidosis

Dr Sampurna Roy MD  

 

                                                                                                                      

 

Sarcoidosis is a relatively common disease of unknown etiology, characterized by multiple, uniform, discrete, noncaseating granulomas in almost any organ of the body.

Visit: Cutaneous Sarcoidosis

The lymphnodes and the lung are most commonly involved.

Visit:  Infectious Granuloma of the Lung  ; Pathological Diagnosis of Granulomatous Lung Diseases ; Non-necrotising Granulomatous Inflammation of the lung ; An approach to Histopathological examination of Pulmonary Granulomatous Inflammation 

Involvement of various organs:

Lymphnodes : Lymphnodes are virtually always involved, most commonly in the hilar and mediastinal regions. Tonsils are affected in about 25 % of the cases.

Spleen and liver : The spleen and liver are microscopically affected in up to 75 % of patients. Splenomegaly occurs in only 18 % of cases. Hepatomegaly is less frequent.

Skin : Cutaneous Sarcoidosis  Cutaneous sarcoid occurs in 30 to 50 % of patients, presenting in a variety of gross forms, including discrete subcutaneous nodules, erythematous scaling plaques and lesions in the mucous membranes.

Eye : The eyes are affected in 20 to 50 % of cases, as iritis, iridocyclitis or choroid retinitis.

 

In pulmonary sarcoidosis radiologically, there is a diffuse reticulonodular infiltrate, but in occasional cases larger nodules are present, a situation referred to as nodular or alveolar sarcoidosis.

Pulmonary sarcoidosis is apparent by lung and hilar lymph node involvement, lymph node enlargement alone or lung disease.

Microscopic features:

Histologically, involvement of both lung and lymph nodes occurs in nearly all instances.

Multiple sarcoid nodules are scattered in the interstitium, particularly in relation to lymphatics, that is, in the bronchovascular bundle and in the lobular septa.

There is increased cellularity of the alveolar walls, owing to an infiltrate of mononuclear cells.

The central part of the granuloma is fibrotic and surrounded by palisaded histiocytes. 

In about 60 % cases, Schaumann bodies (laminated calcified proteinaceous concretions) and asteroid bodies (stellate inclusions within giant cells) are found.

Giant cells at the periphery resemble those of the tuberculosis.

The cuff of lymphocytes that surrounds the histiocytes is inconspicuous, compared with tuberculosis , hence the term “naked tubercle”.

Granulomas also occur in the airways and occasionally may be so prominent as to lead to airway obstruction (endobronchial sarcoid).

Pulmonary lesions have strong tendency to heal, so that only hyalinized scars may be seen microscopically.

The effects on pulmonary function are highly variable.

There may be no abnormalities, classic restrictive lung disease, chronic airflow obstruction, or a mixture of restrictive and obstructive lung disease.

Clinical presentation:

Women are more commonly affected.

Sarcoidosis may be asymptomatic and diagnosed at autopsy or as bilateral hilar adenopathy on a chest x-ray taken for other reasons.

The patients may present as isolated cutaneous or ocular lesions, peripheral lymphadenopathy or hepatospenomegaly, with the insidious onset of respiratory difficulties or constitutional symptoms (fever, night sweats, weight loss), or with an acute onset accompanied by fever, erythema nodosum and polyarthritis.

Hypercalcemia and small lytic lesions in bones, particularly of the hand, may be observed.

Diagnosis of sarcoidosis is one of exclusion of other similar diseases with non-caseating granulomas (Example. tuberculosis, berylliosis, fungal infections).

Clinically, the lesion is slowly progressive, pursue a remitting or resolving course and spontaneously resolved (with or without steroid therapy).

Most patient with sarcoidosis respond well to treatment with corticosteroids or spontaneously remit.

However, some progress to end stage lung disease with honeycombing.

In 65 to 70 % of patients, there are no or only minimal residual manifestations, 20 % have permanent lung or ocular dysfunction, and 10 % die, primarily from progressive pulmonary fibrosis and cor pulmonale.

Diagnosis is established from:

(i) Elevated IgG and calcium ;

(ii) Characteristic chest and phalangeal x-ray findings  ;

(iii) Typical clinical history  ;

(iv) Definite diagnosis is established only by biopsy (often liver or lymphnode) showing non-caseating granulomas.

 

Laboratory  tests used to diagnose sarcoidosis include subcutaneous injection of Kveim antigen (an extract of spleen from a patient with sarcoidosis) and measurements of the serum level of angiotensin converting enzyme.

About 6 weeks after injection of the Kveim antigen, an indurated granulomatous nodule appears in the skin.

The test is seldom used , since the material is scarce.

It is also derived from human material and may therefore by hazardous.

It is now apparent that the raised serum angiotensin converting enzyme is nonspecific and may occur in many interstitial lung diseases.

However it is useful for monitoring the activity of sarcoidosis and the effects of treatment.

Most physicians now use bronchoalveolar lavage to monitor the cell content.

The most reliable procedure for the diagnosis of sarcoidosis is transbronchial lung biopsy, a procedure in which lung tissue is obtained through a fiberoptic  bronchoscope.

Occasionally, biopsy of a mediastinal lymph node by mediastinoscopy is diagnostic.

Etiology:

The distinctive granulomatous respone suggests an immune-mediated phenomenon. Immune abnormalities are as follows:

1.  Lymphocytic alveolitis with numerous CD4+ T cells and activated macrophages.

2.  Cutaneous anergy to wide variety of agents normally induce a local cutaneous delayed hypersensitivity reaction (Example: Tuberculosis).

3. Absolute lymphopenia, mainly due to reduced circulation of T-cells. B lymphocytes are normal in number and are hyperactive.

4. Activated helper T-cells and their secreted cytokines accounts for the influx of monocytes and subsequent granulomas and cell-mediated injury in tissues.

5. Like mycobacterium, there is elevated gamma and delta T-cells and positive polymerase chain reaction assays for mycobacterial DNA.

This has received interest in an infectious cause but the cause of sarcoidosis remains unknown.

 

 

External Link: granuloma.homestead.com

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Further reading:

Cutaneous and pulmonary sarcoidosis in a patient with HIV after highly active antiretroviral therapy.

Practical issues and challenges in the diagnosis and treatment of pulmonary sarcoidosis.

Characterization of sarcoidosis cases in an interstitial lung diseases consultation.

Pulmonary sarcoidosis associated with psoriasis vulgaris: coincidental occurrence or causal association? Case report.

Clinical and radiographic indices associated with airflow limitation in patients with sarcoidosis.

Different angiogenic activity in pulmonary sarcoidosis and idiopathic pulmonary fibrosis.

Disseminated sporotrichosis mimicking sarcoidosis.

Endobronchial biopsy in the diagnosis of pulmonary sarcoidosis.

Expression of CCX CKR in pulmonary sarcoidosis.

Endobronchial biopsy for sarcoidosis: a prospective study.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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